wikidoc - User contributions [en]

Focal segmental glomerulosclerosis overview

2020-05-22T03:03:57Z

Ayesha A. Khan:

<div style="-webkit-user-select: none;">
{|class="infobox" style="position: fixed; top: 65%; right: 10px; margin: 0 0 0 0; border: 0; float: right;
|-
| {{#ev:youtube|https://https://www.youtube.com/watch?v=l7ZyAmGA98w|350}}
|-
|}
__NOTOC__
{{Focal segmental glomerulosclerosis}}
{{CMG}}{{APM}}; '''Associate Editor-In-Chief:’’’[[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com] {{OO}} {{CZ}}

==Overview==

'''Focal segmental glomerulosclerosis''' (FSGS) is a cause of [[nephrotic syndrome]] in children and adolescents, as well as an important cause of [[kidney failure]] in adults.<ref name=Robbins_2005>{{cite book | author = Kumar V, Fausto N, Abbas A (editors) | title = Robbins & Cotran Pathologic Basis of Disease | edition = 7th | pages= pp. 982-3 | publisher = Saunders | year = 2003 | id = ISBN 978-0-721-60187-8 }}</ref> Focal Segmental Glomerulosclerosis (FSGS) is a distinct finding and a descriptive report on the glomerular morphology seen in certain glomerular disease conditions. It is considered to be a glomerular podocytopathy <ref name="pmidPMID: 24589721">{{cite journal| author=Sethi S, Glassock RJ, Fervenza FC| title=Focal segmental glomerulosclerosis: towards a better understanding for the practicing nephrologist. | journal=Nephrol Dial Transplant | year= 2015 | volume= 30 | issue= 3 | pages= 375-84 | pmid=PMID: 24589721 | doi=10.1093/ndt/gfu035 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24589721 }} </ref>. [[Minimal change disease]] (MCD) is by far the most common cause of nephrotic syndrome in children: MCD and primary FSGS may have a similar cause.<ref name=Robbins_2005 /> Focal Segmental Glomerulosclerosis (FSGS) is very similar in presentation to Minimal Change Kidney Disease (MCD), in-fact both are podocytopathies but they are histological different with varying disease signs and symptoms.

The individual components of the name refer to the appearance of the kidney tissue on [[biopsy]]: ''focal'' - only some of the [[glomerulus|glomeruli]] are involved (as opposed to diffuse), ''segmental'' - only part of an entire glomerulus is involved (as opposed to global), ''glomerulosclerosis'' - refers to scarring of the [[glomerulus]] (a part of the [[nephron]] (the functional unit of the [[kidney]])).

FSGS is a progressive form of renal disease, it has become the most common cause of GN-related ESRD in patients with End Stage Renal Disease (ESRD) in the United States. <ref name="pmid15492947">{{cite journal| author=Kitiyakara C, Eggers P, Kopp JB| title=Twenty-one-year trend in ESRD due to focal segmental glomerulosclerosis in the United States. | journal=Am J Kidney Dis | year= 2004 | volume= 44 | issue= 5 | pages= 815-25 | pmid=15492947 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15492947 }} </ref> It accounts for about 40% of adults and about 20% of pediatric cases of Nephrotic Syndrome in the United States. <ref name="pmid15492947">{{cite journal| author=Kitiyakara C, Eggers P, Kopp JB| title=Twenty-one-year trend in ESRD due to focal segmental glomerulosclerosis in the United States. | journal=Am J Kidney Dis | year= 2004 | volume= 44 | issue= 5 | pages= 815-25 | pmid=15492947 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15492947 }} </ref>

FSGS can be a primary or secondary cause of Nephrotic Syndrome. Primary FSGS can occur as an epithelial cell disorder characterized by podocytopathy of the glomeruli that may be related etiologically to minimal change disease. Secondary FSGS occurs due to previous glomerular injury or as a response to previous nephron loss from toxic effects of drugs, viral infections and chronic systemic diseases like diabetes mellitus, SLE, and other renal affecting autoimmune diseases.

Definitive diagnosis of FSGS is by kidney biopsy. High clinical suspicion is also very important in the diagnosis and differentiation of FSGS from MCD. MCD often presents with features of Nephrotic Syndrome like proteinuria, edema and hyperlipidemia while FSGS may present with hematuria, hypertension and decreased renal function which are common presentations of nephritic syndrome.
==Historical Perspective==
[[Focal segmental glomerulosclerosis]] (FSGS) was first discovered by a Theodor Fahr, a German [[pathologist]], in 1925, and he referred to it as "[[lipoid nephrosis]] with [[degeneration]]", showing a clear association to [[minimal change disease]]. In 1957, FSGS was then described by Dr. Arnold Rich, a pathologist at Johns Hopkins University.

==Classification==
FSGS can be classified as primary and secondary [[disease]] depending on [[etiology]], the course of the [[disease]] and [[histologic]] pattern.

==Pathophysiology==
The [[pathophysiology]] of focal segmental glomerulosclerosis (FSGS) is based on two types of FSGS. Primary FSGS is also known as [[idiopathic]] FSGS, there is a [[hypothesis]] that suggests it occurs as a result of circulating [[immune]] activating factors interacting with the [[glomerular]] [[epithelium]]. The underlying [[pathogenesis]] of FSGS is fusion or [[effacement]] of the foot processes ([[podocytes]]) of the [[glomeruli]] and sclerosing of some parts of the [[glomeruli]]. These changes result in [[apoptosis]] and detachment of the [[Glomerular basement membrane|glomerular basement membrane (GBM)]] resulting in subsequent loss of negative charge on [[podocytes]] and podocytopenia. Secondary FSGS is based on [[glomerular]] [[hypertrophy]] and hyperfiltration and over expression of [[inflammatory]] mediators such as, [[TGF-beta]], [[PDGF]] and [[VEGF]]. The underlying [[pathogenesis]] can be based on multiple [[genetic]] [[mutations]] in NPHS1, NEPH1, [[NPHS2]], [[WT1]] and [[INF2]] [[genes]]. Conditions associated with [[FSGS]] include, [[diabetes]], [[HIV]], [[sickle cell disease]], [[nephrotic syndrome]] and [[minimal change disease]]. On [[microscopic]] [[histopathological]] analysis progressive changes seen are, foot process [[effacement]], [[podocyte]] [[apoptosis]], exposed [[GBM]], [[capillary]] expansion and mesangial [[matrix]] [[proliferation]].

==Causes==
==Differentials==
==Risk Factors==
==Screening==
==Epidemiology and Demographics==
==Natural History, Complications, and Prognosis==
==Diagnosis==
===History and Symptoms===
===Physical Examination===
===Laboratory findings===
===Imaging Findings===
===Other Diagnostic Studies===
==Treatment==
===Medical Therapy===
===Surgery===
===Prevention===
==References==
{{Reflist|2}}

{{WH}}
{{WS}}
[[Category:Up-To-Date]]
[[Category:Primary care]]
[[Category:Medicine]]
[[Category:Nephrology]]

Nephrotic syndrome overview

2020-05-22T03:01:57Z

Ayesha A. Khan:

__NOTOC__
{{Nephrotic syndrome}}

{{CMG}} {{APM}} {{AE}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com] {{OO}}

==Overview==
Nephrotic syndrome is group of signs and symptoms resulting from loss of [[kidney]] filtration capabilities leading to massive loss of [[protein]] in urine, generalized or localized body [[edema]], [[Hyperlipoproteinemia|hyperlipidemia]] and [[hypoproteinemia]] (most importantly, [[hypoalbuminemia]]). Causes of Nephrotic Syndrome can be primary (idiopathic) or secondary (from a systemic insult or immune mediated). Nephrotic syndrome (nephrosis) is defined as heavy [[proteinuria]] > 3.5 grams per 24 hours in adults. In children, nephrotic syndrome is defined as protein excretion > 40 mg/m2/h. The accurate diagnosis of nephrotic syndrome thus requires 24-hour urine collection. However, in clinical practice, urine dipstick of a qualitative measure of 3+ urinary [[Protein|proteins]], or spot urine protein (mg)/creatinine(mg) ratio > 2 may also reflect nephrotic syndrome.

== Historical perspective ==
In 1484, Cornelus Roelans of Belgium described a child with “whole body [[swelling]]” and nephropathy. In 1905, Müller described the term of "nephrosis" for non-inflammatory kidney diseases.

==Classification==
Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology. Primary (idiopathic) nephrotic syndrome is defined as nephrotic syndrome due to a primary [[glomerular]] disease. Secondary nephrotic syndrome is defined as nephrotic syndrome due to a primary etiology other than glomerular disorders, such as [[infection]]s, [[malignancies]], systemic conditions, and [[medication]]s.

==Pathophysiology==
The pathophysiology of [[hypoalbuminemia]] in nephrotic syndrome is multifactorial. [[Proteinuria]] plays an important role in the pathogenesis of [[hyperlipidemia]] in nephrotic syndrome. Neurohormonal changes in the [[renin-angiotensin-aldosterone system]], [[vasopressin]], [[atrial natriuretic peptide]] ([[ANP]]), and [[sympathetic nervous system]] are is implicated in [[edema]] formation in nephrotic syndrome.

==Causes==
Nephrotic syndrome can occur primarily or due to systemic diseases. The most common cause of nephrotic syndrome in children is [[minimal change disease]]. The most common primary causes in adults are [[focal segmental glomerulosclerosis]] (FSGS), [[minimal change disease]], and [[membranous nephropathy]]. Approximately 30 percent of adults have secondary nephrotic syndrome due to [[diabetes mellitus]], [[SLE]], or [[amyloidosis]]. The most common cause of secondary nephrotic syndrome in adults is [[diabetes mellitus]].

==Differential Diagnosis==
Nephrotic syndrome should be differentiate from other causes of [[glomerular]] disease such as [[Fabry's disease]], [[post-streptococcal glomerulonephritis]], [[lupus nephritis]], [[Goodpasture syndrome|antiglomerular basement membrane disease]] [[Goodpasture syndrome|(goodpasture's syndrome)]], [[Cryoglobulinemia]], [[Henoch-Schönlein purpura]], [[amyloidosis]], pulmonary-renal syndromes ([[vasculitis]]), [[thin basement membrane disease]], [[Alport syndrome|Alport's Syndrome]], [[Goodpasture syndrome|anti-GBM Disease]], [[hypertensive nephrosclerosis]], and [[subacute bacterial endocarditis]]. The various types of glomerular diseases may be differentiated from each other based on associations, presence of [[pitting edema]], [[hematuria]], [[hypertension]], [[hemoptysis]], [[oliguria]], peri-orbital [[edema]], [[hyperlipidemia]], type of [[antibodies]], [[Light microscope|light]], and [[Electron microscopy|electron microscopic]] features.

==Epidemiology and Demographics==
Idiopathic nephrotic syndrome has an incidence of 2-7 cases per 100,000 and a prevalence of 16 cases per 100,000. Nephrotic syndrome may affect children and adults alike. There is no age or ethnic predominance. The prevalence of nephrotic syndrome in children has a 2 to 1 male to female ratio.

==Natural History, Complications and Prognosis==
Complications of nephrotic syndrome include [[infection]]s, [[thrombotic]] events, and [[renal failure]]. Mortality and overall prognosis depends on the occurrence of complications and adherence to medications.

==Diagnosis==
===History and Symptoms===
The hallmark of nephrotic syndrome is [[edema]]. A positive history of renal disease, systemic diseases such as [[diabetes mellitus]], [[amyloidosis]], or [[systemic lupus erythematosus]], and medication use is suggestive of nephrotic syndrome. The most common symptoms of nephrotic syndrome include volume overload, foamy urine, and [[fatigue]].

===Physical Examination===
A full physical examination should be performed among patients presenting with nephrotic syndrome. Findings on physical examination suggestive of secondary etiologies may be present, such as characteristic [[rash]] in [[systemic lupus erythematosus]] (SLE), or [[peripheral neuropathy]] in [[diabetes mellitus]].

===Laboratory Findings===
Nephrotic syndrome is characterized by the following laboratory findings: [[proteinuria]] > 3.5g/24 hrs on 24-hour urine collection, [[proteinuria]] on urine dipstick, and urine protein/creatinine ratio > 3. When nephrotic syndrome is diagnosed ([[proteinuria]] > 3.5 g/24 hrs), additional laboratory tests are required such as [[serum albumin]] concentration, serum chemistry panel, lipid panel, and serum [[creatinine]] concentration.

===Chest X-Ray===
Chest X-ray may show signs of [[pleural effusion]].

===Ultrasound===
Renal and abdominal doppler ultrasound may be required to investigate for renal etiologies and complications of disease, such as [[renal vein thrombosis]]. Kidney size and signs of obstruction during assessment are also important. [[Doppler ultrasound]] of the extremities is indicated if patients with nephrotic syndrome present with suspected [[deep vein thrombosis]].

===Other Imaging Findings===
[[Renal vein thrombosis]], a complication of nephrotic syndrome, may require any of [[venography]], [[CT scan]], or [[MRI]] for appropriate diagnosis.

===Biopsy===
[[Ultrasound]]-guided renal biopsy for visualization under [[light microscopy]], [[immunofluorescence]] or immunoperoxidase, and [[electron microscopy]] is usually recommended for patients with nephrotic syndrome. Renal biopsy provides diagnostic and prognostic benefit. However, guidelines that define the timing and the circumstances to perform renal [[biopsy]] are not present. In [[minimal change disease]], the most common primary cause of nephrotic syndrome in children, and in [[diabetic nephropathy]], the most common secondary cause of nephrotic syndrome in adults, renal biopsy is not generally recommended and is not routinely performed. Nonetheless, patients who present with unknown or unsure etiology of nephrotic syndrome are recommended to undergo renal biopsy for definitive diagnosis.

==Treatment==

===Medical Therapy===
There are currently no guidelines for the management of [[edema]] associated with nephrotic syndrome. The slow reversal of [[edema]] is important at a rate of 0.5-1 kg daily to prevent [[electrolyte disturbance]]s, [[hypotension]], ischemic [[acute tubular necrosis]], and hemoconcentration associated with aggressive [[diuretic]] therapy. Since [[proteinuria]] is one of the most significant factors for progression of a disease and is associated with outcome, treatment of [[proteinuria]] in nephrotic syndrome must always be considered a priority. [[Angiotensin-converting enzyme inhibitors]] ([[ACE inhibitor|ACE-I]]), with or without [[angiotensin-II receptor blocker]]s ([[ARB]]) have been extensively studied and are well-known to decrease [[proteinuria]] and the risk of progression of [[renal]] disease in patients with nephrotic syndrome. [[Pneumococcal vaccine]]s are recommended for all patients with nephrotic syndrome.

===Surgery===
The mainstay of treatment for nephrotic syndrome is medical therapy.

===Primary Prevention===
Appropriate treatment of conditions that can cause nephrotic syndrome may help prevent the syndrome.

===Secondary Prevention===

[[Category:Kidney diseases]]
[[Category:Nephrology]]
[[Category:Pediatrics]]
[[Category:Disease]]
[[Category:Syndromes]]
[[Category:Primary care]]
{{WH}}
{{WS}}

Goodpasture syndrome overview

2020-05-22T02:59:44Z

Ayesha A. Khan:

Cryoglobulinemia overview

2020-05-22T02:57:41Z

Ayesha A. Khan:

__NOTOC__
{{Cryoglobulinemia}}

{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com]

==Overview==
'''Cryoglobulinemia''' is the presence of high amount of heavy [[globulin]]s (e.g. [[IgM]]) in the [[bloodstream]] which thicken or gel on exposure to cold. Cryoglobulins are circulating [[immunoglobulins]] or [[protein]]s that become insoluble at less than 4 degrees Celsius. The reaction is reversible; redissolution occurs at 37 degrees Celsius. Such proteins are called [[cryoglobulin]]s. Cryoglobulinemia can lead to a medium-sized vessel [[vasculitis]] due to vascular deposition of circulating [[immune complexes]]. This leads to the triad of palpable [[purpura]], [[arthralgia]]s and [[peripheral neuropathy]]. The relationship of cryoglobulins and [[hepatitis C]] infection as well as B cell neoplasia provides an interesting link between infection, autoimmune disease and lymphoproliferative disorders.

==Classification==

==Pathophysiology==

==Causes==

==Differentiating cryoglobulinemia from other Diseases==

==Epidemiology and Demographics==

==Risk Factors==

==Natural History, Complications and Prognosis==

==Diagnosis==

===History and Symptoms===

===Physical Examination===

===Laboratory Findings===

===X Ray===

===CT===

===MRI===

===Ultrasound===

===Other Imaging Findings===

===Other Diagnostic Findings===

==Treatment==

===Medical Therapy===

===Surgery===

===Primary Prevention===

===Secondary Prevention===

==References==
{{Reflist|2}}

[[Category:Disease]]
[[Category:Hematology]]
[[Category:Rheumatology]]
[[Category:Blood tests]]

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

IgA nephropathy overview

2020-05-22T02:55:41Z

Ayesha A. Khan:

<div style="-webkit-user-select: none;">
{| class="infobox" style="position: fixed; top: 65%; right: 10px; margin: 0 0 0 0; border: 0; float: right;"
|-
| {{#ev:youtube|https://https://www.youtube.com/watch?v=794YzMoIc28|350}}
|-
|}
__NOTOC__
{{IgA nephropathy }}
{{CMG}}{{APM}} {{AE}} {{SH}} {{OO}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com]
==Overview==

IgA nephropathy (Berger’s disease) is considered the most common primary [[chronic glomerulonephritis]]. IgA nephropathy is defined immune-histologically by mesangial deposits of [[IgA]], often accompanied by less intense staining for [[IgM]] and/or [[IgG]] and [[Complement system|C3]], in the absence of a systemic disease. IgA nephropathy has been differentiated from [[Henoch-Schönlein purpura|Henoch- Schönlein purpura (HSP)]], which is clearly a systemic illness with [[vasculitis]]. The clinical syndrome of IgA nephropathy is often unpredictable, although classically it is recognized as a [[nephritic syndrome]] with a presentation of recurrent painless [[Hematuria|gross hematuria]] following a [[Respiratory tract infection|respiratory]] or gastrointestinal tract infection in a young male patient. Nonetheless, asymptomatic IgA nephropathy with [[microscopic hematuria]] is not uncommon. Although not frequently performed, the definitive diagnosis to confirm the clinical suspicion of IgA nephropathy is [[Biopsy|kidney biopsy]] that not only carries diagnostic benefit, but also has prognostic implications. IgA nephropathy is a progressive kidney disease that often leads to [[ESRD|End Stage Renal Disease (ESRD)]] due to lack of specific treatments or therapies for this disease. IgA Nephropathy is diagnosed by [[electron microscopy]] of a kidney biopsy specimen showing immunological deposits of predominantly glycosylated but non-galactosed linked IgA1 in the [[mesangium]] of the [[Glomeruli|kidney glomeruli]]. These [[Immune complexes|IgA immune complexes]] deposit comprises of mainly glycosylated [[IgA|immunoglobulin A1 (IgA)]] with some [[Complement 3A|complement C3]] and immunoglobulins G/M ([[IgG]]/ [[IgM]]).

==Historical Perspective==
[[IgA nephropathy]] (Berger disease) was first described by Jean Berger, a pathologist, and Nicole Hinglais, an electron microscopist, in 1968 in France.

==Classification==
IgA nephropathy may be classified according to its association to other pathology or by its histological features. When [[IgA nephropathy]] occurs in isolation, it is called "primary IgA nephropathy". In converse, if IgA nephropathy is a consequence of a more systemic disease, it is called "secondary IgA nephropathy". Additionally, [[IgA nephropathy]] may be histologically classified according to the '''oxford classification''' of [[IgA nephropathy]] as [[Mesangium|mesangial hypercellularity]], [[Glomerulosclerosis|segmental glomerulosclerosis]], endocapillary hypercellularity, or tubular atrophy/interstitial fibrosis.

==Pathophysiology==
[[IgA nephropathy]] is characterized by the presence of aberrant [[IgA|IgA1 immunoglobulins]] deposited on the [[Mesangium|glomerular mesangium]]. [[IgG]] and [[IgM]] may also be present to a much lower extent. On the other hand, serum [[IgA|IgA1]] levels are elevated in patients with [[IgA nephropathy]] in 30-50% of cases. [[IgA|IgA1]] subtypes contain galactose-deficient 3-6 O-glycans that may act as binding sites for anti-N-acetyl-galactosamine antibodies. These antibodies have been shown to be expressed following [[antigenic]] exposure to certain infectious agents. Currently, [[IgA nephropathy]] is believed to be a 4-hit process that eventually leads to [[IgA]] deposition on [[Mesangium|glomerular mesangium]]. Although mesangial deposition is most commonly seen in patients with [[IgA nephropathy]], other pathological features might still be present.

==Causes==
The cause of primary [[IgA nephropathy]] is unknown. Additionally, there are no known infectious or environmental associated factors. However, [[IgA nephropathy]] is associated with some [[genetic mutations]] and familial clustering as a postulated cause of primary [[IgAN]]. [[Liver cirrhosis]], [[celiac disease]], [[HIV]] infection are the most common etiologies associated with glomerular [[IgA]] deposits and thus secondary [[IgA nephropathy]].

==Epidemiology and Demographics==
IgA nephropathy is currently the most common cause of [[Glomerulonephritis|primary glomerulonephritis]] globally, and it is the most common primary [[chronic glomerulonephritis]] in the developed world. IgA nephropathy comprises approximately 10% of all biopsy-proven glomerulonephritis in the USA, 20% of those in Europe and 40-50% of those in Asia. The [[Biopsy|kidney biopsies]] are not routinely performed for all patients with kidney diseases; hence, IgA nephropathy is perhaps under-diagnosed, and its true prevalence remains unknown.
==Risk Factors==
Several risk factors have been found to be associated with IgA nephropathy, most of which seem to be associated with disease outcome and progression into [[ESRD]] rather than disease development. Male gender, native Americans and American and European populations around the pacific rim and asian populations such as China and Japan are more commonly diagnosed with IgA nephropathy.

==Screening==
According to the National Kidney Foundation guidelines for [[glomerulonephritis]], [[screening]] is currently not recommended for IgA nephropathy.

==Natural History, Complications, and Prognosis==
The clinical course of IgA nephropathy varies widely between patients. Although it is generally regarded as a benign disease, emerging data has shown that progression to [[ESRD]] and death are more common than originally believed. Some patients rapidly progress into [[ESRD]]; but the majority experience a stable [[kidney function]] following diagnosis. Commonly, the progression of IgA nephropathy is slower than other notorious glomerular disease. Approximately 20-30% of patients with IgA nephropathy progress to ESRD after 10 years and up to 30-50% of patients develop ESRD over 20 years.

==Diagnosis==
===Diagnostic Study of Choice===

===History and Symptoms===
The majority of patients with IgA nephropathy are asymptomatic. Some patients with IgA nephropathy may develop intermittent gross [[hematuria]] which is often termed as synpharyngitic [[hematuria]], because it occurs after the episodes of [[Bacteria|bacterial]] [[tonsillitis]] or [[Virus|viral]] [[Upper respiratory tract infection|URTI's]]. The patient may also have a positive history of [[flank pain]], [[Low-grade fever|low grade fever]].

===Physical Examination===
Patients with IgA nephropathy usually appear normal and usually have no significant clinical finding upon physical examination. However, some of the patients may present with low-grade [[fever]], [[Hypertension|high blood pressure]] with normal [[pulse pressure]], and pitting [[edema]] of the lower extremities in the late stage if the patient develops [[ESRD]].

===Laboratory Findings===
There are no specific and sensitive diagnostic laboratory findings associated with IgA nephropathy. However all patients with biopsy-proven IgA nephropathy are assessed for secondary causes to rule out common causes of secondary IgA nephropathy. The viral serologies for [[Human Immunodeficiency Virus (HIV)|HIV]], [[HBV]], [[HCV]], [[liver function tests]], and [[electrophoresis]] of [[serum]] [[Antibody|immunoglobulins]] are performed. [[Blood pressure]] measurement, [[Creatinine|serum creatinine]] to estimate [[glomerular filtration rate]] , [[proteinuria]], and pathological features are monitored to assess the risk of progression of the disease.

===Electrocardiogram===
There are no [[ECG]] findings associated with IgA nephropathy.

===X-ray===
There are no [[x-ray]] findings associated with IgA nephropathy.

===Echocardiography and Ultrasound===
For an adult patient with isolated [[hematuria]], [[Medical ultrasonography|ultrasound]] of the kidney is usually done first to pinpoint the source of the [[bleeding]]. The ultrasonography would rule out [[kidney stones]] and [[bladder cancer]], which are the two other common [[urology|urological]] causes of [[hematuria]].

===CT scan===
There are no [[Computed tomography|CT scan]] findings associated with IgA nephropathy.

===MRI===
There are no [[Magnetic resonance imaging|MRI]] findings associated with IgA nephropathy.

===Other Imaging Findings===
There are no other imaging findings associated with IgA nephropathy.
===Other Diagnostic Studies===
For an adult patient with isolated [[hematuria]], diagnostic studies such as [[Medical ultrasonography|ultrasound]] of the kidney and [[cystoscopy]] are usually done first to pinpoint the source of the [[bleeding]]. These diagnostic studies would rule out [[kidney stones]] and [[bladder cancer]], two other common [[urology|urological]] causes of [[hematuria]].

==Treatment==
===Medical Therapy===

===Surgery===
The mainstay of treatment for IgA nephropathy is medical therapy. [[Tonsillectomy]] is usually reserved for patients with recurrent [[Infection|infections]] and renal transplant in patients with [[ESRD]] due to IgA nephropathy and renal transplantation in patients with [[ESRD]] due to IgA nephropathy.
===Primary Prevention===
There are no established measures for the primary prevention of IgA nephropathy.
===Secondary Prevention===
There are no established measures for the secondary prevention of IgA nephropathy.

==References==
{{reflist|2}}
{{WH}}
{{WS}}

[[Category:Nephrology]]
[[Category:Genetic disorders]]

Post-streptococcal glomerulonephritis overview

2020-05-17T21:09:56Z

Ayesha A. Khan:

__NOTOC__
{{Post-streptococcal glomerulonephritis}}

{{CMG}}; {{AE}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com] {{MKK}}
==Overview==
Poststreptococcal glomerulonephritis (PSGN) is caused by preceding infection with nephritogenic strains of group A beta-hemolytic streptococcus. The intial clinical presentation of PSGN is usually asymptomatic then it progresses to microscopic hematuria, proteinuria , edema, hypertension, and symptoms of acute kidney injury. Common risk factors in the development of [[post-streptococcal glomerulonephritis]] include [[streptococcal]] throat infection and [[impetigo]]. Less common risk factors are household infection with the nephritogenic strain of group A streptococcal. Common complications of [[post-streptococcal glomerulonephritis]] include severe [[nephritis]], renal failure , atypical [[hemolytic-uremic syndrome|hemolytic uremic syndrome ,]] refractory hypoxic [[respiratory failure]], and [[seizure|seizures]]. Prognosis is generally excellent but depends upon age and co-morbidities. Laboratory findings consistent with the diagnosis of [[streptococcal infection]] include [[antistreptolysin O]] (ASO) positive, antinicotinamide adenine dinucleotides positive, antihyaluronidase, and anti–DNAse B positive. Other abnormal laboratory findings include [[leukocytosis]] with [[neutrophilia]], [[c-reactive protein|CRP]] is raised, increased levels of [[blood urea nitrogen]] (BUN) and [[serum creatinine]] levels are increased. On serologic testing, [[hypocomplementemia]] is usually found. On urinalysis, [[proteinuria]], [[hematuria]], and dysmorphic red cells are usually found. Effective measures for the primary prevention of [[post-streptococcal glomerulonephritis]] include improving hand hygiene, better housing, prevent overcrowding, treatment of an infected patient within 24 hours with [[antibiotics]] and prevent close contact. A 26-valent vaccine is recommended for children to prevent post-streptococcal glomerulonephritis. Effective measures for the secondary prevention of post-streptococcal glomerulonephritis include compliant with anti-hypertensive medication and follow up with the [[nephrologist]].

==Historical Perspective==
Klebs and colleagues showed that the clinical findings were consistent with a form of [[glomerulonephritis]] that was associated with the period following [[scarlet fever]]. In 1812, Wells showed that a latent period was required for [[edema]] and red urine seem to be present. In 1905 and 1933, Reichel and Osman further elaborated on PSGN and revealed detailed findings and description of the disease, its prevalence, its clinical findings, and its outcome, respectively. In 1903 when Clemens von Pirquet hypothesized the presence of [[immune complexes]] that might be the culprit of PSGN. In 1941, Seegal and Earle determined the nephritogenic properties of [[Streptococcal Infection|streptococcal]] strains and differentiated [[Streptococcal infections|streptococcal]] strains based on their nephritogenic vs. [[rheumatic]] complication. The mainstay of treatment is pharmacotherapy, however dietary therapy is useful for controlling [[edema]] and [[hypertension]]. Dietary therapy includes low [[salt]], [[protein]] intake, and water restriction. If the [[Streptococcal infections|streptococcal]] infection is still present, it should be treated with [[antibiotics]]. To control severe [[hypertension]], [[labetalol]] is usually used, For mild to moderate hypertension, [[furosemide]] is used. For rapidly progressive crescentic acute post-streptococcal glomerulonephritis, [[methylprednisolone]] is preferred

==Classification==
There is no established system for the classification of post-streptococcal glomerulonephritis.

==Pathophysiology==
It is thought that [[post-streptococcal glomerulonephritis]] (PSGN) is caused by nephritogenic strains of group A beta-hemolytic streptococcus (GAS). The mechanism which leads to [[Immunological|immunologic]] injury to the [[glomerulus]] include deposition of [[immune complexes]] with [[streptococcal]] antigenic components, then [[immune complexes]] are deposited in [[glomerular basement membrane]] and antibodies bind to the [[GBM]]. In-situ formation of immune complexes is a characteristic associated with cationic antigens that have a charge-facilitated penetration through the polyanionic glomerular basement membrane. The plasmin-binding capacity of streptococcal antigens favors immune complex deposition and inflammation. The typical pathological changes are endocapillary proliferation with varying degrees of leukocyte infiltration, and C3, IgG, and IgM immune deposits. Electron microscopy shows the hallmark lesion of subepithelial electron dense deposits (“humps”). The immediate prognosis is excellent in children, but adults have a significant early mortality, which partially results from cardiovascular disease.

==Causes==
Common causes of [[post-streptococcal glomerulonephritis]] include infection with group A [[streptococci]]. Others strain of [[streptococci]] which cause [[post-streptococcal glomerulonephritis]] include [[streptococci]] M types 47, 49, 55, 2, 60, and 57 causes pyodermatitis and [[streptococci]] M types 1, 2, 4, 3, 25, 49, and 12 causes [[throat]] [[infection]]. Less common causes of [[post-streptococcal glomerulonephritis]] include group C such as S. zooepidemicus and group G [[Streptococcus|streptococcal]] infections.

==Differentiating Xyz from Other Diseases==
Post-streptococcal glomerulonephritis should be differentiate from other causes of glomerular disease such as [[nephritic syndrome]], [[nephrotic syndrome]], [[Fabry's disease]], [[poststreptococcal glomerulonephritis]], [[lupus nephritis]], [[Goodpasture syndrome|antiglomerular basement membrane disease]] [[Goodpasture syndrome|(goodpasture's syndrome)]], [[Cryoglobulinemia]], [[Henoch-Schönlein purpura]], [[amyloidosis]], pulmonary-renal syndromes ([[vasculitis]]), [[thin basement membrane disease]], [[Alport syndrome|Alport's Syndrome]], [[Goodpasture syndrome|anti-GBM Disease]], [[hypertensive nephrosclerosis]], and [[subacute bacterial endocarditis]]. The various types of glomerular diseases may be differentiated from each other based on associations, presence of [[pitting edema]], hemeturia, [[hypertension]], [[hemoptysis]], [[oliguria]], peri-orbital edema, [[hyperlipidemia]], type of [[antibodies]], [[Light microscope|light]] and [[Electron microscopy|electron microscopic]] features.

==Epidemiology and Demographics==
The incidence of [[post-streptococcal glomerulonephritis]] is approximately 9.5 to 28.5 per 100,000 individuals worldwide. The case-fatality rate of [[post-streptococcal glomerulonephritis]] is approximately 2 percent in India and 0.08 percent in Turkey. It commonly affects children with age between 5 to 12 years. The incidence of [[post-streptococcal glomerulonephritis]] increases in older people age greater than 60 years. It commonly affects children with age between 5 to 12 years. Men are more commonly affected by [[post-streptococcal glomerulonephritis]] than women. The majority of [[post-streptococcal glomerulonephritis]] cases are reported in developing countries.

==Risk Factors==
Common risk factors in the development of [[post-streptococcal glomerulonephritis]] include [[streptococcal]] throat infection and [[impetigo]]. Less common risk factors are household infection with the nephritogenic strain of group A streptococcal.

==Screening==
There is insufficient evidence to recommend routine screening for [[post-streptococcal glomerulonephritis]].

==Natural History, Complications, and Prognosis==
The symptoms of post-streptococcal [[glomerulonephritis]] typically develop one to three weeks after exposure to [[Group A streptococcal infection|group A streptococcal]] throat infection and 3 to 6 weeks after [[Group A streptococcal infection|group A streptococcal]] skin infection. Common complications of [[post-streptococcal glomerulonephritis]] include severe [[nephritis]], renal failure , atypical [[hemolytic-uremic syndrome|hemolytic uremic syndrome ,]] refractory hypoxic [[respiratory failure]], and [[seizure|seizures]]. Prognosis is generally excellent but depends upon age and co-morbidities.

==Diagnosis==
==Diagnostic Study of Choice==
The [[antistreptolysin O]] (ASO) positive is the gold standard test for the diagnosis of post-streptococcal glomerulonephritis.

==History and Symptoms==
Patients with [[post-streptococcal glomerulonephritis]] may have a positive history of [[streptococcal infections|streptococcal]] throat infection and streptococcal skin infection. Common symptoms of post-streptococcal glomerulonephritis include dark [[urine]], [[oliguria]], [[periorbital edema]] and [[hypertension]]. Less common symptoms of post-streptococcal glomerulonephritis include general malaise, [[weakness]], [[anorexia]], [[nausea]] and [[vomiting]].

==Physical Examination==
Patients with post-streptococcal glomerulonephritis usually appear [[lethargic]]. On physical examination, patients usually have high [[blood pressure]], [[periorbital edema]] and [[edema]] of extremities.

==Laboratory Findings==
Laboratory findings consistent with the diagnosis of [[streptococcal infection]] include [[antistreptolysin O]] (ASO) positive, antinicotinamide adenine dinucleotides positive, antihyaluronidase, and anti–DNAse B positive. Other abnormal laboratory findings include [[leukocytosis]] with [[neutrophilia]], [[c-reactive protein|CRP]] is raised, increased levels of [[blood urea nitrogen]] (BUN) and [[serum creatinine]] levels are increased. On serologic testing, [[hypocomplementemia]] is usually found. On urinalysis, [[proteinuria]], [[hematuria]], and dysmorphic red cells are usually found.

==Electrocardiogram==
There are no ECG findings associated with post-streptococcal glomerulonephritis.

==X-ray==
There are no x-ray findings associated with post-streptococcal glomerulonephritis.

==Echocardiography and Ultrasound==
There are no echocardiography/ultrasound findings associated with post-streptococcal glomerulonephritis.

==CT scan==
There are no CT scan findings associated with [[post-streptococcal glomerulonephritis]].

==MRI==
There are no MRI findings associated with [[post-streptococcal glomerulonephritis]].

==Other Imaging Findings==
There are no other imaging findings associated with post-streptococcal glomerulonephritis.

==Other Diagnostic Studies==
Renal biopsy is routinely not done to diagnose [[post-streptococcal glomerulonephritis]]. There are the indications for biopsy include persistent [[proteinuria]] of more than 6 months, persistent microscopic [[hematuria]] more than 18 months, decreasing [[GFR]] after 4 weeks, and persistent [[hypocomplementemia]] after 6 weeks.

==Treatment==
==Medical Therapy==
The mainstay of treatment is pharmacotherapy, however dietary therapy is useful for controlling [[edema]] and [[hypertension]]. Dietary therapy includes low [[salt]], [[protein]] intake, and water restriction. If the [[Streptococcal infections|streptococcal]] infection is still present, it should be treated with [[antibiotics]]. To control severe [[hypertension]], [[labetalol]] is usually used, For mild to moderate hypertension, [[furosemide]] is used. For rapidly progressive crescentic acute post-streptococcal glomerulonephritis, [[methylprednisolone]] is preferred.

==Surgery==
Surgical intervention is not recommended for the management of post-streptococcal glomerulonephritis.

==Primary Prevention==
Effective measures for the primary prevention of [[post-streptococcal glomerulonephritis]] include improving hand hygiene, better housing, prevent overcrowding, treatment of an infected patient within 24 hours with [[antibiotics]] and prevent close contact. A 26-valent vaccine is recommended for children to prevent post-streptococcal glomerulonephritis.

==Secondary Prevention==
Post-streptococcal glomerulonephritis is resolved completely, however, effective measures for the secondary prevention of post-streptococcal glomerulonephritis include compliant with anti-hypertensive medication and follow up with the [[nephrologist]].

==References==

{{Reflist|2}}

[[Category:Kidney diseases]]
[[Category:Need content]]

{{WH}}
{{WS}}

Post-streptococcal glomerulonephritis overview

2020-05-17T21:08:11Z

Ayesha A. Khan: /* Pathophysiology */

__NOTOC__
{{Post-streptococcal glomerulonephritis}}

{{CMG}}; {{AE}} {{MKK}}
==Overview==
Poststreptococcal glomerulonephritis (PSGN) is caused by preceding infection with nephritogenic strains of group A beta-hemolytic streptococcus. The intial clinical presentation of PSGN is usually asymptomatic then it progresses to microscopic hematuria, proteinuria , edema, hypertension, and symptoms of acute kidney injury. Common risk factors in the development of [[post-streptococcal glomerulonephritis]] include [[streptococcal]] throat infection and [[impetigo]]. Less common risk factors are household infection with the nephritogenic strain of group A streptococcal. Common complications of [[post-streptococcal glomerulonephritis]] include severe [[nephritis]], renal failure , atypical [[hemolytic-uremic syndrome|hemolytic uremic syndrome ,]] refractory hypoxic [[respiratory failure]], and [[seizure|seizures]]. Prognosis is generally excellent but depends upon age and co-morbidities. Laboratory findings consistent with the diagnosis of [[streptococcal infection]] include [[antistreptolysin O]] (ASO) positive, antinicotinamide adenine dinucleotides positive, antihyaluronidase, and anti–DNAse B positive. Other abnormal laboratory findings include [[leukocytosis]] with [[neutrophilia]], [[c-reactive protein|CRP]] is raised, increased levels of [[blood urea nitrogen]] (BUN) and [[serum creatinine]] levels are increased. On serologic testing, [[hypocomplementemia]] is usually found. On urinalysis, [[proteinuria]], [[hematuria]], and dysmorphic red cells are usually found. Effective measures for the primary prevention of [[post-streptococcal glomerulonephritis]] include improving hand hygiene, better housing, prevent overcrowding, treatment of an infected patient within 24 hours with [[antibiotics]] and prevent close contact. A 26-valent vaccine is recommended for children to prevent post-streptococcal glomerulonephritis. Effective measures for the secondary prevention of post-streptococcal glomerulonephritis include compliant with anti-hypertensive medication and follow up with the [[nephrologist]].

==Historical Perspective==
Klebs and colleagues showed that the clinical findings were consistent with a form of [[glomerulonephritis]] that was associated with the period following [[scarlet fever]]. In 1812, Wells showed that a latent period was required for [[edema]] and red urine seem to be present. In 1905 and 1933, Reichel and Osman further elaborated on PSGN and revealed detailed findings and description of the disease, its prevalence, its clinical findings, and its outcome, respectively. In 1903 when Clemens von Pirquet hypothesized the presence of [[immune complexes]] that might be the culprit of PSGN. In 1941, Seegal and Earle determined the nephritogenic properties of [[Streptococcal Infection|streptococcal]] strains and differentiated [[Streptococcal infections|streptococcal]] strains based on their nephritogenic vs. [[rheumatic]] complication. The mainstay of treatment is pharmacotherapy, however dietary therapy is useful for controlling [[edema]] and [[hypertension]]. Dietary therapy includes low [[salt]], [[protein]] intake, and water restriction. If the [[Streptococcal infections|streptococcal]] infection is still present, it should be treated with [[antibiotics]]. To control severe [[hypertension]], [[labetalol]] is usually used, For mild to moderate hypertension, [[furosemide]] is used. For rapidly progressive crescentic acute post-streptococcal glomerulonephritis, [[methylprednisolone]] is preferred

==Classification==
There is no established system for the classification of post-streptococcal glomerulonephritis.

==Pathophysiology==
It is thought that [[post-streptococcal glomerulonephritis]] (PSGN) is caused by nephritogenic strains of group A beta-hemolytic streptococcus (GAS). The mechanism which leads to [[Immunological|immunologic]] injury to the [[glomerulus]] include deposition of [[immune complexes]] with [[streptococcal]] antigenic components, then [[immune complexes]] are deposited in [[glomerular basement membrane]] and antibodies bind to the [[GBM]]. In-situ formation of immune complexes is a characteristic associated with cationic antigens that have a charge-facilitated penetration through the polyanionic glomerular basement membrane. The plasmin-binding capacity of streptococcal antigens favors immune complex deposition and inflammation. The typical pathological changes are endocapillary proliferation with varying degrees of leukocyte infiltration, and C3, IgG, and IgM immune deposits. Electron microscopy shows the hallmark lesion of subepithelial electron dense deposits (“humps”). The immediate prognosis is excellent in children, but adults have a significant early mortality, which partially results from cardiovascular disease.

==Causes==
Common causes of [[post-streptococcal glomerulonephritis]] include infection with group A [[streptococci]]. Others strain of [[streptococci]] which cause [[post-streptococcal glomerulonephritis]] include [[streptococci]] M types 47, 49, 55, 2, 60, and 57 causes pyodermatitis and [[streptococci]] M types 1, 2, 4, 3, 25, 49, and 12 causes [[throat]] [[infection]]. Less common causes of [[post-streptococcal glomerulonephritis]] include group C such as S. zooepidemicus and group G [[Streptococcus|streptococcal]] infections.

==Differentiating Xyz from Other Diseases==
Post-streptococcal glomerulonephritis should be differentiate from other causes of glomerular disease such as [[nephritic syndrome]], [[nephrotic syndrome]], [[Fabry's disease]], [[poststreptococcal glomerulonephritis]], [[lupus nephritis]], [[Goodpasture syndrome|antiglomerular basement membrane disease]] [[Goodpasture syndrome|(goodpasture's syndrome)]], [[Cryoglobulinemia]], [[Henoch-Schönlein purpura]], [[amyloidosis]], pulmonary-renal syndromes ([[vasculitis]]), [[thin basement membrane disease]], [[Alport syndrome|Alport's Syndrome]], [[Goodpasture syndrome|anti-GBM Disease]], [[hypertensive nephrosclerosis]], and [[subacute bacterial endocarditis]]. The various types of glomerular diseases may be differentiated from each other based on associations, presence of [[pitting edema]], hemeturia, [[hypertension]], [[hemoptysis]], [[oliguria]], peri-orbital edema, [[hyperlipidemia]], type of [[antibodies]], [[Light microscope|light]] and [[Electron microscopy|electron microscopic]] features.

==Epidemiology and Demographics==
The incidence of [[post-streptococcal glomerulonephritis]] is approximately 9.5 to 28.5 per 100,000 individuals worldwide. The case-fatality rate of [[post-streptococcal glomerulonephritis]] is approximately 2 percent in India and 0.08 percent in Turkey. It commonly affects children with age between 5 to 12 years. The incidence of [[post-streptococcal glomerulonephritis]] increases in older people age greater than 60 years. It commonly affects children with age between 5 to 12 years. Men are more commonly affected by [[post-streptococcal glomerulonephritis]] than women. The majority of [[post-streptococcal glomerulonephritis]] cases are reported in developing countries.

==Risk Factors==
Common risk factors in the development of [[post-streptococcal glomerulonephritis]] include [[streptococcal]] throat infection and [[impetigo]]. Less common risk factors are household infection with the nephritogenic strain of group A streptococcal.

==Screening==
There is insufficient evidence to recommend routine screening for [[post-streptococcal glomerulonephritis]].

==Natural History, Complications, and Prognosis==
The symptoms of post-streptococcal [[glomerulonephritis]] typically develop one to three weeks after exposure to [[Group A streptococcal infection|group A streptococcal]] throat infection and 3 to 6 weeks after [[Group A streptococcal infection|group A streptococcal]] skin infection. Common complications of [[post-streptococcal glomerulonephritis]] include severe [[nephritis]], renal failure , atypical [[hemolytic-uremic syndrome|hemolytic uremic syndrome ,]] refractory hypoxic [[respiratory failure]], and [[seizure|seizures]]. Prognosis is generally excellent but depends upon age and co-morbidities.

==Diagnosis==
==Diagnostic Study of Choice==
The [[antistreptolysin O]] (ASO) positive is the gold standard test for the diagnosis of post-streptococcal glomerulonephritis.

==History and Symptoms==
Patients with [[post-streptococcal glomerulonephritis]] may have a positive history of [[streptococcal infections|streptococcal]] throat infection and streptococcal skin infection. Common symptoms of post-streptococcal glomerulonephritis include dark [[urine]], [[oliguria]], [[periorbital edema]] and [[hypertension]]. Less common symptoms of post-streptococcal glomerulonephritis include general malaise, [[weakness]], [[anorexia]], [[nausea]] and [[vomiting]].

==Physical Examination==
Patients with post-streptococcal glomerulonephritis usually appear [[lethargic]]. On physical examination, patients usually have high [[blood pressure]], [[periorbital edema]] and [[edema]] of extremities.

==Laboratory Findings==
Laboratory findings consistent with the diagnosis of [[streptococcal infection]] include [[antistreptolysin O]] (ASO) positive, antinicotinamide adenine dinucleotides positive, antihyaluronidase, and anti–DNAse B positive. Other abnormal laboratory findings include [[leukocytosis]] with [[neutrophilia]], [[c-reactive protein|CRP]] is raised, increased levels of [[blood urea nitrogen]] (BUN) and [[serum creatinine]] levels are increased. On serologic testing, [[hypocomplementemia]] is usually found. On urinalysis, [[proteinuria]], [[hematuria]], and dysmorphic red cells are usually found.

==Electrocardiogram==
There are no ECG findings associated with post-streptococcal glomerulonephritis.

==X-ray==
There are no x-ray findings associated with post-streptococcal glomerulonephritis.

==Echocardiography and Ultrasound==
There are no echocardiography/ultrasound findings associated with post-streptococcal glomerulonephritis.

==CT scan==
There are no CT scan findings associated with [[post-streptococcal glomerulonephritis]].

==MRI==
There are no MRI findings associated with [[post-streptococcal glomerulonephritis]].

==Other Imaging Findings==
There are no other imaging findings associated with post-streptococcal glomerulonephritis.

==Other Diagnostic Studies==
Renal biopsy is routinely not done to diagnose [[post-streptococcal glomerulonephritis]]. There are the indications for biopsy include persistent [[proteinuria]] of more than 6 months, persistent microscopic [[hematuria]] more than 18 months, decreasing [[GFR]] after 4 weeks, and persistent [[hypocomplementemia]] after 6 weeks.

==Treatment==
==Medical Therapy==
The mainstay of treatment is pharmacotherapy, however dietary therapy is useful for controlling [[edema]] and [[hypertension]]. Dietary therapy includes low [[salt]], [[protein]] intake, and water restriction. If the [[Streptococcal infections|streptococcal]] infection is still present, it should be treated with [[antibiotics]]. To control severe [[hypertension]], [[labetalol]] is usually used, For mild to moderate hypertension, [[furosemide]] is used. For rapidly progressive crescentic acute post-streptococcal glomerulonephritis, [[methylprednisolone]] is preferred.

==Surgery==
Surgical intervention is not recommended for the management of post-streptococcal glomerulonephritis.

==Primary Prevention==
Effective measures for the primary prevention of [[post-streptococcal glomerulonephritis]] include improving hand hygiene, better housing, prevent overcrowding, treatment of an infected patient within 24 hours with [[antibiotics]] and prevent close contact. A 26-valent vaccine is recommended for children to prevent post-streptococcal glomerulonephritis.

==Secondary Prevention==
Post-streptococcal glomerulonephritis is resolved completely, however, effective measures for the secondary prevention of post-streptococcal glomerulonephritis include compliant with anti-hypertensive medication and follow up with the [[nephrologist]].

==References==

{{Reflist|2}}

[[Category:Kidney diseases]]
[[Category:Need content]]

{{WH}}
{{WS}}

Acute tubular necrosis overview

2020-05-17T20:08:18Z

Ayesha A. Khan:

__NOTOC__
{{Acute tubular necrosis}}
{{CMG}}; {{AE}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com] {{CK}}

==Overview==

Acute tubular necrosis (ATN) defines a pathologic process rather than a clinical syndrome in which varying degrees of renal tubular injury occur. Clinically, ATN manifests as acute kidney injury although the terms have previously been used interchangeably. ATN is the most common cause of overt AKI. Despite the term, ATN does not necessarily imply cellular necrosis with evidence of non-necrotic injury observed more consistently. Furthermore, clinicopathologic correlation is often irrelevant with severe renal insufficiency sometimes seen with modest pathological findings.<ref name="pmid18235086">{{cite journal| author=Rosen S, Stillman IE| title=Acute tubular necrosis is a syndrome of physiologic and pathologic dissociation. | journal=J Am Soc Nephrol | year= 2008 | volume= 19 | issue= 5 | pages= 871-5 | pmid=18235086 | doi=10.1681/ASN.2007080913 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18235086 }} </ref> ATN can be either ischemic or toxin induced. Classically, ischemic ATN follows hypotension or hypovolemia with patchy involvement usually observed. On the other hand, toxic ATN is usually a dose-dependent injury seen with medications, diagnostic agents, and heavy metals with proximal tubule damage involving almost all nephrons.<ref name="Fogo">Fogo A, Cohen AH, Colvin RB et al. Fundamentals of Renal Pathology. Springer 2013. Acute Tubular Necrosis. http://dx.doi.org/10.1007/978-3-642-39080-7_15 </ref>

==Historical Perspective==
Eric and beallduring was the first to publish a article on describing ATN in detail during world war II. In 1938, Councilman was the first to discover the association between systemic infections and the development of ATN.

==Classification==
Acute tubular necrosis may be classified based on mechanisms of [[Renal tubule|tubular]] [[injury]] into three categories ischemic, toxin-induced, and mixed.

==Pathophysiology==
Classically the course of ischemic ATN has been divided into 3 phases: Initiation, maintenance, and recovery. During the initiation phase, immediately following the insult, sublethal cellular injury occurs, with loss of cell polarity and brush border. The maintenance phase is reached after the irreversible renal parenchymal injury has been established. During the last 2 phases, both tubular cell death and cell regeneration occur simultaneously. Apoptosis has been reported in the initial phase of acute tubular necrosis and during the recovery phase. With initial ischemic or cytotoxic injury, a number of tubular cells may undergo apoptosis. The ET-1 gene has also been shown to be upregulated during ischemic injuries. When exposed to ischemic stress, tubular cells are prone to loss polarity and even detachment of viable cells due to the disruption of key structural anchors. Several important proteins are required for tubular cells to maintain their structure and polarity including the actin cytoskeleton, microvilli, and junctional complexes such as tight junctions and adherens junctions. The most common cause of acute kidney injury (AKI) is acute tubular necrosis (ATN) when the pattern of injury lies within the kidney (intrinsic disease). The term tubular necrosis is a misnomer, as true cellular necrosis is usually minimal, and the alteration is not limited to the tubular structures. Acute tubular necrosis is most common in hospitalized patients and is associated with high morbidity and mortality. The pattern of injury that defines acute tubular necrosis includes renal tubular cell damage and death. Intrarenal vasoconstriction or a direct effect of drug toxicity is caused by an ischemic event, nephrotoxic mechanism, or a mixture of both.

==Causes==
Acute tubular necrosis is commonly caused by [[Kidney|renal]] [[ischemia]] resulting from conditions such as [[Hypovolemia|volume depletion]], [[hypotension]], [[Sepsis|septic shock]], [[cirrhosis]], and [[Disseminated intravascular coagulation|DIC]]. It is also caused by exposure to various nephrotoxic medications including [[Aminoglycoside|aminoglycosides]], [[amphotericin B]], [[ACE inhibitor|ACE inhibitors]], [[Non-steroidal anti-inflammatory drug|NSAIDs]], antiviral drugs, [[Chemotherapy|cytotoxic therapy]],and also exposure to [[Contrast media|radio contrast substances]].

==Differentiating Acute tubular necrosis from Other Diseases==

==Epidemiology and Demographics==
[[Incidence]] of acute tubular necrosis is approximately 88 per 100,000 individuals worldwide. The mean age at [[diagnosis]] of acute tubular necrosis was 59.5 years. [[Mortality rate]] is high with acute tubular necrosis among hospitalized and [[Intensive care unit|ICU]] patients. Acute tubular necrosis affects men and women equally.

==Risk Factors==
Common risk factors in the development of acute tubular necrosis include any condition that lead to decreased renal perfusion such as recent abdominal and cardiac surgery, marked [[hypovolemia]], [[sepsis]], [[hemorrhagic shock]], severe [[pancreatitis]], and [[diabetes mellitus]]. Nephrotoxic medications ( eg, [[ACE inhibitor|ACE inhibitors]], [[Non-steroidal anti-inflammatory drug|NSAIDs]], [[Aminoglycoside|aminoglycosides]], radio [[Contrast medium|contrast media]]) can also be a risk for developing acute tubular necrosis.

==Screening==
[[Screening (medicine)|Screening]] for acute tubular necrosis is usually not recommended for asymptomatic individuals. [[Screening (medicine)|Screening]] is usually recommended for [[Patient|patients]] who are at high risk for developing acute tubular necrosis. Screening evaluation includes measurement of serum [[creatinine]], urine output, [[blood urea nitrogen]], urinary and serum electrolytes.

==Natural History, Complications, and Prognosis==

Acute tubular necrosis may usually develop through 3 phases, initiation, maintenance and recovery. Common complications of acute tubular necrosis include [[Electrolyte disturbance|electrolyte imbalance]](eg, [[hyperkalemia]], [[hyperphosphatemia]], [[hypocalcemia]], and [[metabolic acidosis]]), [[platelet]] dysfunction and altered [[consciousness]] or [[coma]]. [[Prognosis]] depends on the underlying [[etiology]] and severity of [[kidney]] damage. When compared to ischemic acute tubular necrosis, nephrotoxic and mixed acute tubular necrosis have the good [[prognosis]].
==Diagnosis==
===Diagnostic Study of Choice===
There is no single [[diagnostic study of choice]] for the [[diagnosis]] of acute tubular necrosis, but acute tubular necrosis can be diagnosed based on serum [[creatinine]] and [[Blood urea nitrogen|BUN]] levels, [[urinalysis]], urine [[Electrolyte|electrolytes]] (urine [[sodium]], [[Fractional excretion of sodium|fractional excretion of sodium concentration]]), and [[Medical ultrasonography|ultrasonography]] with [[Doppler|doppler imaging]].

===History and Symptoms===
History taking is an important aspect in making a [[diagnosis]] of acute tubular necrosis. It provides clues to precipitating factors, [[Etiology|causes]] and associated [[Comorbidity|comorbid]] conditions leading to decreased [[Kidney|renal]] perfusion and [[kidney]] injury. Most common [[Symptom|symptoms]] of acute tubular necrosis include decreased or absent urinary output, [[Dizziness|postural dizziness]], [[edema]], excess [[thirst]], [[tachycardia]], [[altered mental status]] and easy [[Fatigue|fatiguability]].

===Physical Examination===
On [[physical examination]], [[Patient|patients]] with acute tubular necrosis may show the findings of [[Hypovolemia|volume depletion]]. They usually appear [[Ill feeling|ill]], [[Dehydration|dehydrated]], and [[Fatigue|lethargic]]. Common [[physical examination]]<nowiki/>findings of acute tubular necrosis include [[orthostatic hypotension]] and other signs of [[hypovolemia]] (dry [[mucous membranes]], sunken [[Eye|eyes]], poor skin turgor and [[Capillary refill time|delayed capillary refill]], and decreased [[jugular venous pressure]]).

===Laboratory Findings===
[[Complete blood count|CBC]], [[urinalysis]] with sediment [[microscopy]], [[urine]] electrolytes, [[osmolarity]], serum electrolytes, [[blood urea nitrogen]] and [[Creatinine|serum creatinine]], and [[Dipsticks|urine dipstick]] are commonly performed in patients to evaluate acute tubular necrosis and other causes of [[Acute kidney injury|acute renal failure]]. [[Urine]] sediment may show tubular epithelial cells and epithelial cell casts or brown muddy granular [[casts]]. Increased urine [[sodium]] concentration >40 mEq/L, urine [[Fractional sodium excretion|fractional excretion of sodium]] greater than 2 percent along with elevated serum [[creatinine]] concentration at a rate greater than 0.3 mg/dL/day may be found in [[acute tubular necrosis]]. However, these tests may have some limitations.

===Electrocardiogram===
There are no [[The electrocardiogram|ECG]] findings associated with acute tubular necrosis. An [[The electrocardiogram|ECG]] may be helpful in the [[diagnosis]] of [[Electrolyte disturbance|electrolyte imbalance]] occurs as a complication of acute tubular necrosis.

===X-ray===
There are no specific x-ray findings associated with acute tubular necrosis. However, an abdominal x-ray may be helpful in diagnosing renal calculi, and areas of obstruction.

===Echocardiography and Ultrasound===
[[Ultrasound]] with [[Doppler|doppler imaging]] can be helpful in the [[diagnosis]] of acute tubular necrosis. Findings on an [[ultrasound]] include normal or increased [[kidney]] size, alterations in cortical [[echogenicity]]<nowiki/>and increased [[Resistive index|RI]]. There are no [[echocardiography]] findings associated with acute tubular necrosis. However, an [[echocardiography]] may be helpful in the diagnosis of complications of acute tubular necrosis.

===CT scan===
[[Computed tomography|CT scan]] findings of patients with acute tubular necrosis may include alterations in [[kidney]] size, striate nephrogram, accumulation of fluid around [[Kidney|kidneys]]. CT scan can also detect hydronephrosis that cannot be detectable on ultrasound.

===MRI===
There are no specific [[Magnetic resonance imaging|MRI]] findings associated with acute tubular necrosis. MRI may show alteration in [[kidney]] size, outflow obstruction areas that can not be clearly visible on [[ultrasound]].

===Other Imaging Findings===
There are no other imaging findings associated with acute tubular necrosis.

===Other Diagnostic Studies===
Renal [[biopsy]] and detection of various novel biomarkers in the serum and urine can be helpful in diagnosing acute tubular necrosis.

==Treatment==
===Medical Therapy===
According to the Kidney Disease Improving Global Outcomes (KDIGO) 2012 guidelines, management approach of acute tubular necrosis include examination of all patients thoroughly to identify the cause, precipitating factors, and comorbid conditions leading to a rapid reduction in GFR, which may be reversible and regular monitor patients for serum creatinine, BUN, and urine output to assess the severity of renal damage.

===Surgery===
Surgery is not the first-line treatment option for patients with acute tubular necrosis.

===Primary Prevention===
Effective measures for the [[primary prevention]] of acute tubular necrosis include identification of individuals who are at high risk and prompt treatment for underlying conditions, maintain volume status and adequate [[Kidney|renal]] perfusion by proper [[hydration]] or isotonic fluid administration, monitoring fluid intake, urine output and serum [[creatinine]] levels regular intervals to ensure normal renal function and avoiding or decreasing dose of nephrotoxins and [[Contrast medium|contrast media]].

===Secondary Prevention===
Secondary preventive measures of acute tubular necrosis are similar to [[primary prevention]].

==References==
{{reflist|2}}

[[Category:Nephrology]]
[[Category:Disease]]

Acute tubular necrosis overview

2020-05-17T20:07:04Z

Ayesha A. Khan: /* Pathophysiology */

__NOTOC__
{{Acute tubular necrosis}}
{{CMG}}; {{AE}} {{CK}}

==Overview==

Acute tubular necrosis (ATN) defines a pathologic process rather than a clinical syndrome in which varying degrees of renal tubular injury occur. Clinically, ATN manifests as acute kidney injury although the terms have previously been used interchangeably. ATN is the most common cause of overt AKI. Despite the term, ATN does not necessarily imply cellular necrosis with evidence of non-necrotic injury observed more consistently. Furthermore, clinicopathologic correlation is often irrelevant with severe renal insufficiency sometimes seen with modest pathological findings.<ref name="pmid18235086">{{cite journal| author=Rosen S, Stillman IE| title=Acute tubular necrosis is a syndrome of physiologic and pathologic dissociation. | journal=J Am Soc Nephrol | year= 2008 | volume= 19 | issue= 5 | pages= 871-5 | pmid=18235086 | doi=10.1681/ASN.2007080913 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18235086 }} </ref> ATN can be either ischemic or toxin induced. Classically, ischemic ATN follows hypotension or hypovolemia with patchy involvement usually observed. On the other hand, toxic ATN is usually a dose-dependent injury seen with medications, diagnostic agents, and heavy metals with proximal tubule damage involving almost all nephrons.<ref name="Fogo">Fogo A, Cohen AH, Colvin RB et al. Fundamentals of Renal Pathology. Springer 2013. Acute Tubular Necrosis. http://dx.doi.org/10.1007/978-3-642-39080-7_15 </ref>

==Historical Perspective==
Eric and beallduring was the first to publish a article on describing ATN in detail during world war II. In 1938, Councilman was the first to discover the association between systemic infections and the development of ATN.

==Classification==
Acute tubular necrosis may be classified based on mechanisms of [[Renal tubule|tubular]] [[injury]] into three categories ischemic, toxin-induced, and mixed.

==Pathophysiology==
Classically the course of ischemic ATN has been divided into 3 phases: Initiation, maintenance, and recovery. During the initiation phase, immediately following the insult, sublethal cellular injury occurs, with loss of cell polarity and brush border. The maintenance phase is reached after the irreversible renal parenchymal injury has been established. During the last 2 phases, both tubular cell death and cell regeneration occur simultaneously. Apoptosis has been reported in the initial phase of acute tubular necrosis and during the recovery phase. With initial ischemic or cytotoxic injury, a number of tubular cells may undergo apoptosis. The ET-1 gene has also been shown to be upregulated during ischemic injuries. When exposed to ischemic stress, tubular cells are prone to loss polarity and even detachment of viable cells due to the disruption of key structural anchors. Several important proteins are required for tubular cells to maintain their structure and polarity including the actin cytoskeleton, microvilli, and junctional complexes such as tight junctions and adherens junctions. The most common cause of acute kidney injury (AKI) is acute tubular necrosis (ATN) when the pattern of injury lies within the kidney (intrinsic disease). The term tubular necrosis is a misnomer, as true cellular necrosis is usually minimal, and the alteration is not limited to the tubular structures. Acute tubular necrosis is most common in hospitalized patients and is associated with high morbidity and mortality. The pattern of injury that defines acute tubular necrosis includes renal tubular cell damage and death. Intrarenal vasoconstriction or a direct effect of drug toxicity is caused by an ischemic event, nephrotoxic mechanism, or a mixture of both.

==Causes==
Acute tubular necrosis is commonly caused by [[Kidney|renal]] [[ischemia]] resulting from conditions such as [[Hypovolemia|volume depletion]], [[hypotension]], [[Sepsis|septic shock]], [[cirrhosis]], and [[Disseminated intravascular coagulation|DIC]]. It is also caused by exposure to various nephrotoxic medications including [[Aminoglycoside|aminoglycosides]], [[amphotericin B]], [[ACE inhibitor|ACE inhibitors]], [[Non-steroidal anti-inflammatory drug|NSAIDs]], antiviral drugs, [[Chemotherapy|cytotoxic therapy]],and also exposure to [[Contrast media|radio contrast substances]].

==Differentiating Acute tubular necrosis from Other Diseases==

==Epidemiology and Demographics==
[[Incidence]] of acute tubular necrosis is approximately 88 per 100,000 individuals worldwide. The mean age at [[diagnosis]] of acute tubular necrosis was 59.5 years. [[Mortality rate]] is high with acute tubular necrosis among hospitalized and [[Intensive care unit|ICU]] patients. Acute tubular necrosis affects men and women equally.

==Risk Factors==
Common risk factors in the development of acute tubular necrosis include any condition that lead to decreased renal perfusion such as recent abdominal and cardiac surgery, marked [[hypovolemia]], [[sepsis]], [[hemorrhagic shock]], severe [[pancreatitis]], and [[diabetes mellitus]]. Nephrotoxic medications ( eg, [[ACE inhibitor|ACE inhibitors]], [[Non-steroidal anti-inflammatory drug|NSAIDs]], [[Aminoglycoside|aminoglycosides]], radio [[Contrast medium|contrast media]]) can also be a risk for developing acute tubular necrosis.

==Screening==
[[Screening (medicine)|Screening]] for acute tubular necrosis is usually not recommended for asymptomatic individuals. [[Screening (medicine)|Screening]] is usually recommended for [[Patient|patients]] who are at high risk for developing acute tubular necrosis. Screening evaluation includes measurement of serum [[creatinine]], urine output, [[blood urea nitrogen]], urinary and serum electrolytes.

==Natural History, Complications, and Prognosis==

Acute tubular necrosis may usually develop through 3 phases, initiation, maintenance and recovery. Common complications of acute tubular necrosis include [[Electrolyte disturbance|electrolyte imbalance]](eg, [[hyperkalemia]], [[hyperphosphatemia]], [[hypocalcemia]], and [[metabolic acidosis]]), [[platelet]] dysfunction and altered [[consciousness]] or [[coma]]. [[Prognosis]] depends on the underlying [[etiology]] and severity of [[kidney]] damage. When compared to ischemic acute tubular necrosis, nephrotoxic and mixed acute tubular necrosis have the good [[prognosis]].
==Diagnosis==
===Diagnostic Study of Choice===
There is no single [[diagnostic study of choice]] for the [[diagnosis]] of acute tubular necrosis, but acute tubular necrosis can be diagnosed based on serum [[creatinine]] and [[Blood urea nitrogen|BUN]] levels, [[urinalysis]], urine [[Electrolyte|electrolytes]] (urine [[sodium]], [[Fractional excretion of sodium|fractional excretion of sodium concentration]]), and [[Medical ultrasonography|ultrasonography]] with [[Doppler|doppler imaging]].

===History and Symptoms===
History taking is an important aspect in making a [[diagnosis]] of acute tubular necrosis. It provides clues to precipitating factors, [[Etiology|causes]] and associated [[Comorbidity|comorbid]] conditions leading to decreased [[Kidney|renal]] perfusion and [[kidney]] injury. Most common [[Symptom|symptoms]] of acute tubular necrosis include decreased or absent urinary output, [[Dizziness|postural dizziness]], [[edema]], excess [[thirst]], [[tachycardia]], [[altered mental status]] and easy [[Fatigue|fatiguability]].

===Physical Examination===
On [[physical examination]], [[Patient|patients]] with acute tubular necrosis may show the findings of [[Hypovolemia|volume depletion]]. They usually appear [[Ill feeling|ill]], [[Dehydration|dehydrated]], and [[Fatigue|lethargic]]. Common [[physical examination]]<nowiki/>findings of acute tubular necrosis include [[orthostatic hypotension]] and other signs of [[hypovolemia]] (dry [[mucous membranes]], sunken [[Eye|eyes]], poor skin turgor and [[Capillary refill time|delayed capillary refill]], and decreased [[jugular venous pressure]]).

===Laboratory Findings===
[[Complete blood count|CBC]], [[urinalysis]] with sediment [[microscopy]], [[urine]] electrolytes, [[osmolarity]], serum electrolytes, [[blood urea nitrogen]] and [[Creatinine|serum creatinine]], and [[Dipsticks|urine dipstick]] are commonly performed in patients to evaluate acute tubular necrosis and other causes of [[Acute kidney injury|acute renal failure]]. [[Urine]] sediment may show tubular epithelial cells and epithelial cell casts or brown muddy granular [[casts]]. Increased urine [[sodium]] concentration >40 mEq/L, urine [[Fractional sodium excretion|fractional excretion of sodium]] greater than 2 percent along with elevated serum [[creatinine]] concentration at a rate greater than 0.3 mg/dL/day may be found in [[acute tubular necrosis]]. However, these tests may have some limitations.

===Electrocardiogram===
There are no [[The electrocardiogram|ECG]] findings associated with acute tubular necrosis. An [[The electrocardiogram|ECG]] may be helpful in the [[diagnosis]] of [[Electrolyte disturbance|electrolyte imbalance]] occurs as a complication of acute tubular necrosis.

===X-ray===
There are no specific x-ray findings associated with acute tubular necrosis. However, an abdominal x-ray may be helpful in diagnosing renal calculi, and areas of obstruction.

===Echocardiography and Ultrasound===
[[Ultrasound]] with [[Doppler|doppler imaging]] can be helpful in the [[diagnosis]] of acute tubular necrosis. Findings on an [[ultrasound]] include normal or increased [[kidney]] size, alterations in cortical [[echogenicity]]<nowiki/>and increased [[Resistive index|RI]]. There are no [[echocardiography]] findings associated with acute tubular necrosis. However, an [[echocardiography]] may be helpful in the diagnosis of complications of acute tubular necrosis.

===CT scan===
[[Computed tomography|CT scan]] findings of patients with acute tubular necrosis may include alterations in [[kidney]] size, striate nephrogram, accumulation of fluid around [[Kidney|kidneys]]. CT scan can also detect hydronephrosis that cannot be detectable on ultrasound.

===MRI===
There are no specific [[Magnetic resonance imaging|MRI]] findings associated with acute tubular necrosis. MRI may show alteration in [[kidney]] size, outflow obstruction areas that can not be clearly visible on [[ultrasound]].

===Other Imaging Findings===
There are no other imaging findings associated with acute tubular necrosis.

===Other Diagnostic Studies===
Renal [[biopsy]] and detection of various novel biomarkers in the serum and urine can be helpful in diagnosing acute tubular necrosis.

==Treatment==
===Medical Therapy===
According to the Kidney Disease Improving Global Outcomes (KDIGO) 2012 guidelines, management approach of acute tubular necrosis include examination of all patients thoroughly to identify the cause, precipitating factors, and comorbid conditions leading to a rapid reduction in GFR, which may be reversible and regular monitor patients for serum creatinine, BUN, and urine output to assess the severity of renal damage.

===Surgery===
Surgery is not the first-line treatment option for patients with acute tubular necrosis.

===Primary Prevention===
Effective measures for the [[primary prevention]] of acute tubular necrosis include identification of individuals who are at high risk and prompt treatment for underlying conditions, maintain volume status and adequate [[Kidney|renal]] perfusion by proper [[hydration]] or isotonic fluid administration, monitoring fluid intake, urine output and serum [[creatinine]] levels regular intervals to ensure normal renal function and avoiding or decreasing dose of nephrotoxins and [[Contrast medium|contrast media]].

===Secondary Prevention===
Secondary preventive measures of acute tubular necrosis are similar to [[primary prevention]].

==References==
{{reflist|2}}

[[Category:Nephrology]]
[[Category:Disease]]

Acute tubular necrosis overview

2020-05-17T20:06:09Z

Ayesha A. Khan:

__NOTOC__
{{Acute tubular necrosis}}
{{CMG}}; {{AE}} {{CK}}

==Overview==

Acute tubular necrosis (ATN) defines a pathologic process rather than a clinical syndrome in which varying degrees of renal tubular injury occur. Clinically, ATN manifests as acute kidney injury although the terms have previously been used interchangeably. ATN is the most common cause of overt AKI. Despite the term, ATN does not necessarily imply cellular necrosis with evidence of non-necrotic injury observed more consistently. Furthermore, clinicopathologic correlation is often irrelevant with severe renal insufficiency sometimes seen with modest pathological findings.<ref name="pmid18235086">{{cite journal| author=Rosen S, Stillman IE| title=Acute tubular necrosis is a syndrome of physiologic and pathologic dissociation. | journal=J Am Soc Nephrol | year= 2008 | volume= 19 | issue= 5 | pages= 871-5 | pmid=18235086 | doi=10.1681/ASN.2007080913 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18235086 }} </ref> ATN can be either ischemic or toxin induced. Classically, ischemic ATN follows hypotension or hypovolemia with patchy involvement usually observed. On the other hand, toxic ATN is usually a dose-dependent injury seen with medications, diagnostic agents, and heavy metals with proximal tubule damage involving almost all nephrons.<ref name="Fogo">Fogo A, Cohen AH, Colvin RB et al. Fundamentals of Renal Pathology. Springer 2013. Acute Tubular Necrosis. http://dx.doi.org/10.1007/978-3-642-39080-7_15 </ref>

==Historical Perspective==
Eric and beallduring was the first to publish a article on describing ATN in detail during world war II. In 1938, Councilman was the first to discover the association between systemic infections and the development of ATN.

==Classification==
Acute tubular necrosis may be classified based on mechanisms of [[Renal tubule|tubular]] [[injury]] into three categories ischemic, toxin-induced, and mixed.

==Pathophysiology==
Classically the course of ischemic ATN has been divided into 3 phases: Initiation, maintenance, and recovery. During the initiation phase, immediately following the insult, sublethal cellular injury occurs, with loss of cell polarity and brush border. The maintenance phase is reached after the irreversible renal parenchymal injury has been established. During the last 2 phases, both tubular cell death and cell regeneration occur simultaneously. Apoptosis has been reported in the initial phase of acute tubular necrosis and during the recovery phase. With initial ischemic or cytotoxic injury, a number of tubular cells may undergo apoptosis. The ET-1 gene has also been shown to be upregulated during ischemic injuries. When exposed to ischemic stress, tubular cells are prone to loss polarity and even detachment of viable cells due to the disruption of key structural anchors. Several important proteins are required for tubular cells to maintain their structure and polarity including the actin cytoskeleton, microvilli, and junctional complexes such as tight junctions and adherens junctions. The most common cause of acute kidney injury (AKI) is acute tubular necrosis (ATN) when the pattern of injury lies within the kidney (intrinsic disease). The term tubular necrosis is a misnomer, as true cellular necrosis is usually minimal, and the alteration is not limited to the tubular structures. Acute tubular necrosis is most common in hospitalized patients and is associated with high morbidity and mortality. The pattern of injury that defines acute tubular necrosis includes renal tubular cell damage and death. Intrarenal vasoconstriction or a direct effect of drug toxicity is caused by an ischemic event, nephrotoxic mechanism, or a mixture of both
==Causes==
Acute tubular necrosis is commonly caused by [[Kidney|renal]] [[ischemia]] resulting from conditions such as [[Hypovolemia|volume depletion]], [[hypotension]], [[Sepsis|septic shock]], [[cirrhosis]], and [[Disseminated intravascular coagulation|DIC]]. It is also caused by exposure to various nephrotoxic medications including [[Aminoglycoside|aminoglycosides]], [[amphotericin B]], [[ACE inhibitor|ACE inhibitors]], [[Non-steroidal anti-inflammatory drug|NSAIDs]], antiviral drugs, [[Chemotherapy|cytotoxic therapy]],and also exposure to [[Contrast media|radio contrast substances]].

==Differentiating Acute tubular necrosis from Other Diseases==

==Epidemiology and Demographics==
[[Incidence]] of acute tubular necrosis is approximately 88 per 100,000 individuals worldwide. The mean age at [[diagnosis]] of acute tubular necrosis was 59.5 years. [[Mortality rate]] is high with acute tubular necrosis among hospitalized and [[Intensive care unit|ICU]] patients. Acute tubular necrosis affects men and women equally.

==Risk Factors==
Common risk factors in the development of acute tubular necrosis include any condition that lead to decreased renal perfusion such as recent abdominal and cardiac surgery, marked [[hypovolemia]], [[sepsis]], [[hemorrhagic shock]], severe [[pancreatitis]], and [[diabetes mellitus]]. Nephrotoxic medications ( eg, [[ACE inhibitor|ACE inhibitors]], [[Non-steroidal anti-inflammatory drug|NSAIDs]], [[Aminoglycoside|aminoglycosides]], radio [[Contrast medium|contrast media]]) can also be a risk for developing acute tubular necrosis.

==Screening==
[[Screening (medicine)|Screening]] for acute tubular necrosis is usually not recommended for asymptomatic individuals. [[Screening (medicine)|Screening]] is usually recommended for [[Patient|patients]] who are at high risk for developing acute tubular necrosis. Screening evaluation includes measurement of serum [[creatinine]], urine output, [[blood urea nitrogen]], urinary and serum electrolytes.

==Natural History, Complications, and Prognosis==

Acute tubular necrosis may usually develop through 3 phases, initiation, maintenance and recovery. Common complications of acute tubular necrosis include [[Electrolyte disturbance|electrolyte imbalance]](eg, [[hyperkalemia]], [[hyperphosphatemia]], [[hypocalcemia]], and [[metabolic acidosis]]), [[platelet]] dysfunction and altered [[consciousness]] or [[coma]]. [[Prognosis]] depends on the underlying [[etiology]] and severity of [[kidney]] damage. When compared to ischemic acute tubular necrosis, nephrotoxic and mixed acute tubular necrosis have the good [[prognosis]].
==Diagnosis==
===Diagnostic Study of Choice===
There is no single [[diagnostic study of choice]] for the [[diagnosis]] of acute tubular necrosis, but acute tubular necrosis can be diagnosed based on serum [[creatinine]] and [[Blood urea nitrogen|BUN]] levels, [[urinalysis]], urine [[Electrolyte|electrolytes]] (urine [[sodium]], [[Fractional excretion of sodium|fractional excretion of sodium concentration]]), and [[Medical ultrasonography|ultrasonography]] with [[Doppler|doppler imaging]].

===History and Symptoms===
History taking is an important aspect in making a [[diagnosis]] of acute tubular necrosis. It provides clues to precipitating factors, [[Etiology|causes]] and associated [[Comorbidity|comorbid]] conditions leading to decreased [[Kidney|renal]] perfusion and [[kidney]] injury. Most common [[Symptom|symptoms]] of acute tubular necrosis include decreased or absent urinary output, [[Dizziness|postural dizziness]], [[edema]], excess [[thirst]], [[tachycardia]], [[altered mental status]] and easy [[Fatigue|fatiguability]].

===Physical Examination===
On [[physical examination]], [[Patient|patients]] with acute tubular necrosis may show the findings of [[Hypovolemia|volume depletion]]. They usually appear [[Ill feeling|ill]], [[Dehydration|dehydrated]], and [[Fatigue|lethargic]]. Common [[physical examination]]<nowiki/>findings of acute tubular necrosis include [[orthostatic hypotension]] and other signs of [[hypovolemia]] (dry [[mucous membranes]], sunken [[Eye|eyes]], poor skin turgor and [[Capillary refill time|delayed capillary refill]], and decreased [[jugular venous pressure]]).

===Laboratory Findings===
[[Complete blood count|CBC]], [[urinalysis]] with sediment [[microscopy]], [[urine]] electrolytes, [[osmolarity]], serum electrolytes, [[blood urea nitrogen]] and [[Creatinine|serum creatinine]], and [[Dipsticks|urine dipstick]] are commonly performed in patients to evaluate acute tubular necrosis and other causes of [[Acute kidney injury|acute renal failure]]. [[Urine]] sediment may show tubular epithelial cells and epithelial cell casts or brown muddy granular [[casts]]. Increased urine [[sodium]] concentration >40 mEq/L, urine [[Fractional sodium excretion|fractional excretion of sodium]] greater than 2 percent along with elevated serum [[creatinine]] concentration at a rate greater than 0.3 mg/dL/day may be found in [[acute tubular necrosis]]. However, these tests may have some limitations.

===Electrocardiogram===
There are no [[The electrocardiogram|ECG]] findings associated with acute tubular necrosis. An [[The electrocardiogram|ECG]] may be helpful in the [[diagnosis]] of [[Electrolyte disturbance|electrolyte imbalance]] occurs as a complication of acute tubular necrosis.

===X-ray===
There are no specific x-ray findings associated with acute tubular necrosis. However, an abdominal x-ray may be helpful in diagnosing renal calculi, and areas of obstruction.

===Echocardiography and Ultrasound===
[[Ultrasound]] with [[Doppler|doppler imaging]] can be helpful in the [[diagnosis]] of acute tubular necrosis. Findings on an [[ultrasound]] include normal or increased [[kidney]] size, alterations in cortical [[echogenicity]]<nowiki/>and increased [[Resistive index|RI]]. There are no [[echocardiography]] findings associated with acute tubular necrosis. However, an [[echocardiography]] may be helpful in the diagnosis of complications of acute tubular necrosis.

===CT scan===
[[Computed tomography|CT scan]] findings of patients with acute tubular necrosis may include alterations in [[kidney]] size, striate nephrogram, accumulation of fluid around [[Kidney|kidneys]]. CT scan can also detect hydronephrosis that cannot be detectable on ultrasound.

===MRI===
There are no specific [[Magnetic resonance imaging|MRI]] findings associated with acute tubular necrosis. MRI may show alteration in [[kidney]] size, outflow obstruction areas that can not be clearly visible on [[ultrasound]].

===Other Imaging Findings===
There are no other imaging findings associated with acute tubular necrosis.

===Other Diagnostic Studies===
Renal [[biopsy]] and detection of various novel biomarkers in the serum and urine can be helpful in diagnosing acute tubular necrosis.

==Treatment==
===Medical Therapy===
According to the Kidney Disease Improving Global Outcomes (KDIGO) 2012 guidelines, management approach of acute tubular necrosis include examination of all patients thoroughly to identify the cause, precipitating factors, and comorbid conditions leading to a rapid reduction in GFR, which may be reversible and regular monitor patients for serum creatinine, BUN, and urine output to assess the severity of renal damage.

===Surgery===
Surgery is not the first-line treatment option for patients with acute tubular necrosis.

===Primary Prevention===
Effective measures for the [[primary prevention]] of acute tubular necrosis include identification of individuals who are at high risk and prompt treatment for underlying conditions, maintain volume status and adequate [[Kidney|renal]] perfusion by proper [[hydration]] or isotonic fluid administration, monitoring fluid intake, urine output and serum [[creatinine]] levels regular intervals to ensure normal renal function and avoiding or decreasing dose of nephrotoxins and [[Contrast medium|contrast media]].

===Secondary Prevention===
Secondary preventive measures of acute tubular necrosis are similar to [[primary prevention]].

==References==
{{reflist|2}}

[[Category:Nephrology]]
[[Category:Disease]]

Acute renal failure overview

2020-05-17T20:00:56Z

Ayesha A. Khan:

__NOTOC__
{{Acute renal failure}}
{{CMG}} {{AE}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com]

==Overview==
Acute renal failure (ARF), also known as acute kidney failure, is a rapid loss of [[renal function]] due to damage to the [[kidney]]s, resulting in retention of nitrogenous ([[urea]] and [[creatinine]]) and non-nitrogenous waste products that are normally excreted by the kidney. Depending on the severity and duration of the renal dysfunction, this accumulation is accompanied by metabolic disturbances, such as [[metabolic acidosis]] (acidification of the blood) and [[hyperkalaemia]] (elevated potassium levels), changes in body [[fluid balance]], and effects on many other organ systems. It can be characterized by [[oliguria]] or [[anuria]] (decrease or cessation of urine production), although ''nonoliguric ARF'' may occur. It is a serious disease and treated as a [[medical emergency]].
==Historical Perspective==
Before the advancement of modern medicine, acute renal failure might be referred to as uremic poisoning. [[Uremia]] was the term used to describe the contamination of the [[blood]] with [[urine]]. Starting around 1847 this term was used to describe reduced urine output, now known as [[oliguria]], which was thought to be caused by the urine's mixing with the blood instead of being voided through the [[urethra]]. Acute renal failure due to [[acute tubular necrosis]] (ATN) was recognised in the 1940s in the United Kingdom, where crush victims during the Battle of Britain developed patchy necrosis of renal tubules, leading to a sudden decrease in renal function.<ref>{{cite journal |author=Bywaters EG, Beall D |title=Crush injuries with impairment of renal function. |journal=[[British Medical Journal|Br Med J]] |volume= |issue=1 |pages=427-32 |year=1941 |pmid=9527411 |doi= |url=http://jasn.asnjournals.org/cgi/pmidlookup?view=long&pmid=9527411}}</ref> During the Korean and Vietnam wars, the incidence of ARF decreased due to better acute management and intravenous infusion of fluids.<ref>{{cite journal |author=Schrier RW, Wang W, Poole B, Mitra A |title=Acute renal failure: definitions, diagnosis, pathogenesis, and therapy |journal=J. Clin. Invest. |volume=114 |issue=1 |pages=5–14 |year=2004 |pmid=15232604 |pmc=437979 |doi=10.1172/JCI22353 |url=}}</ref>
==Classification==
Acute renal failure can complicate a wide spectrum of disorders, and for the purpose of diagnosis and management is divided according to the mechanism that lead to renal compromise. The three categories are pre-renal [[azotemia]] (diseases that cause renal [[hypoperfusion]]), renal azotemia (diseases directly affecting the renal parenchyma), and post-renal azotemia (diseases affecting the [[urinary tract]] causing [[urinary tract obstruction|obstruction]]). However, the first consensus definition described and the most used definition is known as the RIFLE criteria. The acronym combines a classification of 3 levels of renal dysfunction (Risk, Injury, Failure) with 2 clinical outcomes (Loss, ESRD) to give the RIFLE stages of AKI.

==Pathophysiology==
[[Acute kidney injury]] (AKI) is the leading cause of [[nephrology]] consultation and is associated with high [[mortality rates. The primary causes of [[AKI]] include ischemia, [[hypoxia]] or [[nephrotoxicity]]. An underlying feature is a rapid decline in [[GFR]] usually associated with decreases in [[renal blood flow]]. Inflammation represents an important additional component of AKI leading to the extension phase of injury, which may be associated with insensitivity to vasodilator therapy. It is suggested that targeting the extension phase represents an area potential of treatment with the greatest possible impact. The underlying basis of [[renal]] injury appears to be impaired energetics of the highly metabolically active [[nephron]] segments (i.e., proximal tubules and thick ascending limb) in the [[renal]] outer [[medulla]], which can trigger conversion from transient [[hypoxia]] to intrinsic [[renal failure]]. [[Injury]] to [[kidney]] cells can be lethal or sublethal. Sublethal injury represents an important component in AKI, as it may profoundly influence GFR and renal blood flow. The nature of the recovery response is mediated by the degree to which sublethal cells can restore normal function and promote regeneration. The successful recovery from AKI depends on the degree to which these repair processes ensue and these may be compromised in elderly or CKD patients. Recent data suggest that AKI represents a potential link to CKD in surviving patients. Finally, earlier diagnosis of AKI represents an important area in treating patients with AKI that has spawned increased awareness of the potential that biomarkers of AKI may play in the future.

==Causes==
[[Glomerular filtration rate]] depends on multiple factors, particularly the [[renal blood flow]]. The pressure difference across the renal vasculature i.e the difference in pressure between the renal arterioles and venules is directly proportional to the [[GFR]]. Any decrease in [[renal blood flow]], injury to the renal tubules or obstruction to the urinary tract outflow can cause decreased urinary output.
==Differentiating Acute renal failure from other Diseases==
Various parameters like [[fractional sodium excretion]], urinary sodium concentration, urine osmolality and U/P [[creatinine]] ratio have been used to diagnose acute renal failure. The [[fractional sodium excretion]] is identifies as the most effective non-invasive test in formulating the differential diagnosis of acute renal failure <ref name="pmid7363517">{{cite journal |author=Espinel CH, Gregory AW |title=Differential diagnosis of acute renal failure |journal=[[Clinical Nephrology]] |volume=13 |issue=2 |pages=73–7 |year=1980 |month=February |pmid=7363517 |doi= |url=}}</ref>.
==Epidemiology and Demographics==
Acute kidney injury is common among hospitalized patients. It affects some 3-7% of patients admitted to the hospital and approximately 25-30% of patients in the [[intensive care unit]] <ref name="isbn1-4160-3110-3">{{cite book |author= |title=Brenner and Rector's The Kidney |publisher=Saunders |location=Philadelphia |year=2007 |pages= |isbn=1-4160-3110-3 |oclc= |doi= |accessdate=}}</ref>.
==Risk Factors==
Acute renal failure always occurs in connection with some other medical condition or event. Being hospitalized with a serious condition requiring intensive care is the biggest risk factor. The risk factors for acute renal failure is divided into three major categories, namely pre-renal factors, renal factors and post-renal factors.
==Diagnosis==
===History and Symptoms===
When a patient presents with [[uremia|uremic]] symptoms, first step is to differentiate an acute from a chronic process. A recent rise in serum [[creatinine]] levels indicate an acute pathology of renal failure, whereas chronically elevated [[creatinine]] levels are seen in [[chronic renal failure]]. However, [[chronic renal insufficiency]] is also associated with [[osteopathy]], [[neuropathy]] and small, scarred [[kidney]]s.
===Laboratory Findings===
Renal failure is generally diagnosed either when [[creatinine]] or [[blood urea nitrogen]] tests are markedly elevated in an ill patient, especially when oliguria is present. Previous measurements of renal function may offer comparison, which is especially important if a patient is known to have [[chronic renal failure]] as well. If the cause is not apparent, a large amount of [[blood test]]s and examination of a [[urine]] specimen is typically performed to elucidate the cause of acute renal failure.
===Ultrasound===
[[Medical ultrasonography]] of the renal tract is essential to rule out obstruction of the urinary tract.
===Other Diagnostic Studies===
Kidney [[biopsy]] may be performed in the setting of acute renal failure, to provide a definitive diagnosis and sometimes an idea of the [[prognosis]], unless the cause is clear and appropriate screening investigations are reassuringly negative.
==Treatment==
===Surgery===
If the cause of the acute renal failure is obstruction of the urinary tract, relief of the obstruction (with a [[nephrostomy]] or [[urinary catheter]]) may be necessary. Lack of improvement with fluid resuscitation, therapy-resistant hyperkalemia, metabolic acidosis, or fluid overload may necessitate artificial support in the form of [[dialysis]] or [[hemofiltration]]. Depending on the cause, a proportion of patients will never regain full renal function, thus having [[end stage renal failure]] requiring lifelong [[dialysis]] or a [[kidney transplant]].

==References==

{{Reflist|2}}
{{WH}}
{{WS}}
[[Category:Needs overview]]
[[Category:Medical emergencies]]
[[Category:Kidney diseases]]
[[Category:Organ failure]]
[[Category:Causes of death]]
[[Category:Nephrology]]
[[Category:Emergency medicine]]
[[Category:Intensive care medicine]]

Acute renal failure overview

2020-05-17T19:57:12Z

Ayesha A. Khan:

__NOTOC__
{{Acute renal failure}}
{{CMG}} {{AE}}

==Overview==
Acute renal failure (ARF), also known as acute kidney failure, is a rapid loss of [[renal function]] due to damage to the [[kidney]]s, resulting in retention of nitrogenous ([[urea]] and [[creatinine]]) and non-nitrogenous waste products that are normally excreted by the kidney. Depending on the severity and duration of the renal dysfunction, this accumulation is accompanied by metabolic disturbances, such as [[metabolic acidosis]] (acidification of the blood) and [[hyperkalaemia]] (elevated potassium levels), changes in body [[fluid balance]], and effects on many other organ systems. It can be characterized by [[oliguria]] or [[anuria]] (decrease or cessation of urine production), although ''nonoliguric ARF'' may occur. It is a serious disease and treated as a [[medical emergency]].
==Historical Perspective==
Before the advancement of modern medicine, acute renal failure might be referred to as uremic poisoning. [[Uremia]] was the term used to describe the contamination of the [[blood]] with [[urine]]. Starting around 1847 this term was used to describe reduced urine output, now known as [[oliguria]], which was thought to be caused by the urine's mixing with the blood instead of being voided through the [[urethra]]. Acute renal failure due to [[acute tubular necrosis]] (ATN) was recognised in the 1940s in the United Kingdom, where crush victims during the Battle of Britain developed patchy necrosis of renal tubules, leading to a sudden decrease in renal function.<ref>{{cite journal |author=Bywaters EG, Beall D |title=Crush injuries with impairment of renal function. |journal=[[British Medical Journal|Br Med J]] |volume= |issue=1 |pages=427-32 |year=1941 |pmid=9527411 |doi= |url=http://jasn.asnjournals.org/cgi/pmidlookup?view=long&pmid=9527411}}</ref> During the Korean and Vietnam wars, the incidence of ARF decreased due to better acute management and intravenous infusion of fluids.<ref>{{cite journal |author=Schrier RW, Wang W, Poole B, Mitra A |title=Acute renal failure: definitions, diagnosis, pathogenesis, and therapy |journal=J. Clin. Invest. |volume=114 |issue=1 |pages=5–14 |year=2004 |pmid=15232604 |pmc=437979 |doi=10.1172/JCI22353 |url=}}</ref>
==Classification==
Acute renal failure can complicate a wide spectrum of disorders, and for the purpose of diagnosis and management is divided according to the mechanism that lead to renal compromise. The three categories are pre-renal [[azotemia]] (diseases that cause renal [[hypoperfusion]]), renal azotemia (diseases directly affecting the renal parenchyma), and post-renal azotemia (diseases affecting the [[urinary tract]] causing [[urinary tract obstruction|obstruction]]). However, the first consensus definition described and the most used definition is known as the RIFLE criteria. The acronym combines a classification of 3 levels of renal dysfunction (Risk, Injury, Failure) with 2 clinical outcomes (Loss, ESRD) to give the RIFLE stages of AKI.

==Pathophysiology==
[[Acute kidney injury]] (AKI) is the leading cause of [[nephrology]] consultation and is associated with high [[mortality rates. The primary causes of [[AKI]] include ischemia, [[hypoxia]] or [[nephrotoxicity]]. An underlying feature is a rapid decline in [[GFR]] usually associated with decreases in [[renal blood flow]]. Inflammation represents an important additional component of AKI leading to the extension phase of injury, which may be associated with insensitivity to vasodilator therapy. It is suggested that targeting the extension phase represents an area potential of treatment with the greatest possible impact. The underlying basis of [[renal]] injury appears to be impaired energetics of the highly metabolically active [[nephron]] segments (i.e., proximal tubules and thick ascending limb) in the [[renal]] outer [[medulla]], which can trigger conversion from transient [[hypoxia]] to intrinsic [[renal failure]]. [[Injury]] to [[kidney]] cells can be lethal or sublethal. Sublethal injury represents an important component in AKI, as it may profoundly influence GFR and renal blood flow. The nature of the recovery response is mediated by the degree to which sublethal cells can restore normal function and promote regeneration. The successful recovery from AKI depends on the degree to which these repair processes ensue and these may be compromised in elderly or CKD patients. Recent data suggest that AKI represents a potential link to CKD in surviving patients. Finally, earlier diagnosis of AKI represents an important area in treating patients with AKI that has spawned increased awareness of the potential that biomarkers of AKI may play in the future.

==Causes==
[[Glomerular filtration rate]] depends on multiple factors, particularly the [[renal blood flow]]. The pressure difference across the renal vasculature i.e the difference in pressure between the renal arterioles and venules is directly proportional to the [[GFR]]. Any decrease in [[renal blood flow]], injury to the renal tubules or obstruction to the urinary tract outflow can cause decreased urinary output.
==Differentiating Acute renal failure from other Diseases==
Various parameters like [[fractional sodium excretion]], urinary sodium concentration, urine osmolality and U/P [[creatinine]] ratio have been used to diagnose acute renal failure. The [[fractional sodium excretion]] is identifies as the most effective non-invasive test in formulating the differential diagnosis of acute renal failure <ref name="pmid7363517">{{cite journal |author=Espinel CH, Gregory AW |title=Differential diagnosis of acute renal failure |journal=[[Clinical Nephrology]] |volume=13 |issue=2 |pages=73–7 |year=1980 |month=February |pmid=7363517 |doi= |url=}}</ref>.
==Epidemiology and Demographics==
Acute kidney injury is common among hospitalized patients. It affects some 3-7% of patients admitted to the hospital and approximately 25-30% of patients in the [[intensive care unit]] <ref name="isbn1-4160-3110-3">{{cite book |author= |title=Brenner and Rector's The Kidney |publisher=Saunders |location=Philadelphia |year=2007 |pages= |isbn=1-4160-3110-3 |oclc= |doi= |accessdate=}}</ref>.
==Risk Factors==
Acute renal failure always occurs in connection with some other medical condition or event. Being hospitalized with a serious condition requiring intensive care is the biggest risk factor. The risk factors for acute renal failure is divided into three major categories, namely pre-renal factors, renal factors and post-renal factors.
==Diagnosis==
===History and Symptoms===
When a patient presents with [[uremia|uremic]] symptoms, first step is to differentiate an acute from a chronic process. A recent rise in serum [[creatinine]] levels indicate an acute pathology of renal failure, whereas chronically elevated [[creatinine]] levels are seen in [[chronic renal failure]]. However, [[chronic renal insufficiency]] is also associated with [[osteopathy]], [[neuropathy]] and small, scarred [[kidney]]s.
===Laboratory Findings===
Renal failure is generally diagnosed either when [[creatinine]] or [[blood urea nitrogen]] tests are markedly elevated in an ill patient, especially when oliguria is present. Previous measurements of renal function may offer comparison, which is especially important if a patient is known to have [[chronic renal failure]] as well. If the cause is not apparent, a large amount of [[blood test]]s and examination of a [[urine]] specimen is typically performed to elucidate the cause of acute renal failure.
===Ultrasound===
[[Medical ultrasonography]] of the renal tract is essential to rule out obstruction of the urinary tract.
===Other Diagnostic Studies===
Kidney [[biopsy]] may be performed in the setting of acute renal failure, to provide a definitive diagnosis and sometimes an idea of the [[prognosis]], unless the cause is clear and appropriate screening investigations are reassuringly negative.
==Treatment==
===Surgery===
If the cause of the acute renal failure is obstruction of the urinary tract, relief of the obstruction (with a [[nephrostomy]] or [[urinary catheter]]) may be necessary. Lack of improvement with fluid resuscitation, therapy-resistant hyperkalemia, metabolic acidosis, or fluid overload may necessitate artificial support in the form of [[dialysis]] or [[hemofiltration]]. Depending on the cause, a proportion of patients will never regain full renal function, thus having [[end stage renal failure]] requiring lifelong [[dialysis]] or a [[kidney transplant]].

==References==

{{Reflist|2}}
{{WH}}
{{WS}}
[[Category:Needs overview]]
[[Category:Medical emergencies]]
[[Category:Kidney diseases]]
[[Category:Organ failure]]
[[Category:Causes of death]]
[[Category:Nephrology]]
[[Category:Emergency medicine]]
[[Category:Intensive care medicine]]

Acute renal failure overview

2020-05-17T19:46:56Z

Ayesha A. Khan: /* Pathophysiology */

__NOTOC__
{{Acute renal failure}}
{{CMG}}

==Overview==
Acute renal failure (ARF), also known as acute kidney failure, is a rapid loss of [[renal function]] due to damage to the [[kidney]]s, resulting in retention of nitrogenous ([[urea]] and [[creatinine]]) and non-nitrogenous waste products that are normally excreted by the kidney. Depending on the severity and duration of the renal dysfunction, this accumulation is accompanied by metabolic disturbances, such as [[metabolic acidosis]] (acidification of the blood) and [[hyperkalaemia]] (elevated potassium levels), changes in body [[fluid balance]], and effects on many other organ systems. It can be characterized by [[oliguria]] or [[anuria]] (decrease or cessation of urine production), although ''nonoliguric ARF'' may occur. It is a serious disease and treated as a [[medical emergency]].
==Historical Perspective==
Before the advancement of modern medicine, acute renal failure might be referred to as uremic poisoning. [[Uremia]] was the term used to describe the contamination of the [[blood]] with [[urine]]. Starting around 1847 this term was used to describe reduced urine output, now known as [[oliguria]], which was thought to be caused by the urine's mixing with the blood instead of being voided through the [[urethra]]. Acute renal failure due to [[acute tubular necrosis]] (ATN) was recognised in the 1940s in the United Kingdom, where crush victims during the Battle of Britain developed patchy necrosis of renal tubules, leading to a sudden decrease in renal function.<ref>{{cite journal |author=Bywaters EG, Beall D |title=Crush injuries with impairment of renal function. |journal=[[British Medical Journal|Br Med J]] |volume= |issue=1 |pages=427-32 |year=1941 |pmid=9527411 |doi= |url=http://jasn.asnjournals.org/cgi/pmidlookup?view=long&pmid=9527411}}</ref> During the Korean and Vietnam wars, the incidence of ARF decreased due to better acute management and intravenous infusion of fluids.<ref>{{cite journal |author=Schrier RW, Wang W, Poole B, Mitra A |title=Acute renal failure: definitions, diagnosis, pathogenesis, and therapy |journal=J. Clin. Invest. |volume=114 |issue=1 |pages=5–14 |year=2004 |pmid=15232604 |pmc=437979 |doi=10.1172/JCI22353 |url=}}</ref>
==Classification==
Acute renal failure can complicate a wide spectrum of disorders, and for the purpose of diagnosis and management is divided according to the mechanism that lead to renal compromise. The three categories are pre-renal [[azotemia]] (diseases that cause renal [[hypoperfusion]]), renal azotemia (diseases directly affecting the renal parenchyma), and post-renal azotemia (diseases affecting the [[urinary tract]] causing [[urinary tract obstruction|obstruction]]). However, the first consensus definition described and the most used definition is known as the RIFLE criteria. The acronym combines a classification of 3 levels of renal dysfunction (Risk, Injury, Failure) with 2 clinical outcomes (Loss, ESRD) to give the RIFLE stages of AKI.

==Pathophysiology==
[[Acute kidney injury]] (AKI) is the leading cause of [[nephrology]] consultation and is associated with high [[mortality rates. The primary causes of [[AKI]] include ischemia, [[hypoxia]] or [[nephrotoxicity]]. An underlying feature is a rapid decline in [[GFR]] usually associated with decreases in [[renal blood flow]]. Inflammation represents an important additional component of AKI leading to the extension phase of injury, which may be associated with insensitivity to vasodilator therapy. It is suggested that targeting the extension phase represents an area potential of treatment with the greatest possible impact. The underlying basis of [[renal]] injury appears to be impaired energetics of the highly metabolically active [[nephron]] segments (i.e., proximal tubules and thick ascending limb) in the [[renal]] outer [[medulla]], which can trigger conversion from transient [[hypoxia]] to intrinsic [[renal failure]]. [[Injury]] to [[kidney]] cells can be lethal or sublethal. Sublethal injury represents an important component in AKI, as it may profoundly influence GFR and renal blood flow. The nature of the recovery response is mediated by the degree to which sublethal cells can restore normal function and promote regeneration. The successful recovery from AKI depends on the degree to which these repair processes ensue and these may be compromised in elderly or CKD patients. Recent data suggest that AKI represents a potential link to CKD in surviving patients. Finally, earlier diagnosis of AKI represents an important area in treating patients with AKI that has spawned increased awareness of the potential that biomarkers of AKI may play in the future.

==Causes==
[[Glomerular filtration rate]] depends on multiple factors, particularly the [[renal blood flow]]. The pressure difference across the renal vasculature i.e the difference in pressure between the renal arterioles and venules is directly proportional to the [[GFR]]. Any decrease in [[renal blood flow]], injury to the renal tubules or obstruction to the urinary tract outflow can cause decreased urinary output.
==Differentiating Acute renal failure from other Diseases==
Various parameters like [[fractional sodium excretion]], urinary sodium concentration, urine osmolality and U/P [[creatinine]] ratio have been used to diagnose acute renal failure. The [[fractional sodium excretion]] is identifies as the most effective non-invasive test in formulating the differential diagnosis of acute renal failure <ref name="pmid7363517">{{cite journal |author=Espinel CH, Gregory AW |title=Differential diagnosis of acute renal failure |journal=[[Clinical Nephrology]] |volume=13 |issue=2 |pages=73–7 |year=1980 |month=February |pmid=7363517 |doi= |url=}}</ref>.
==Epidemiology and Demographics==
Acute kidney injury is common among hospitalized patients. It affects some 3-7% of patients admitted to the hospital and approximately 25-30% of patients in the [[intensive care unit]] <ref name="isbn1-4160-3110-3">{{cite book |author= |title=Brenner and Rector's The Kidney |publisher=Saunders |location=Philadelphia |year=2007 |pages= |isbn=1-4160-3110-3 |oclc= |doi= |accessdate=}}</ref>.
==Risk Factors==
Acute renal failure always occurs in connection with some other medical condition or event. Being hospitalized with a serious condition requiring intensive care is the biggest risk factor. The risk factors for acute renal failure is divided into three major categories, namely pre-renal factors, renal factors and post-renal factors.
==Diagnosis==
===History and Symptoms===
When a patient presents with [[uremia|uremic]] symptoms, first step is to differentiate an acute from a chronic process. A recent rise in serum [[creatinine]] levels indicate an acute pathology of renal failure, whereas chronically elevated [[creatinine]] levels are seen in [[chronic renal failure]]. However, [[chronic renal insufficiency]] is also associated with [[osteopathy]], [[neuropathy]] and small, scarred [[kidney]]s.
===Laboratory Findings===
Renal failure is generally diagnosed either when [[creatinine]] or [[blood urea nitrogen]] tests are markedly elevated in an ill patient, especially when oliguria is present. Previous measurements of renal function may offer comparison, which is especially important if a patient is known to have [[chronic renal failure]] as well. If the cause is not apparent, a large amount of [[blood test]]s and examination of a [[urine]] specimen is typically performed to elucidate the cause of acute renal failure.
===Ultrasound===
[[Medical ultrasonography]] of the renal tract is essential to rule out obstruction of the urinary tract.
===Other Diagnostic Studies===
Kidney [[biopsy]] may be performed in the setting of acute renal failure, to provide a definitive diagnosis and sometimes an idea of the [[prognosis]], unless the cause is clear and appropriate screening investigations are reassuringly negative.
==Treatment==
===Surgery===
If the cause of the acute renal failure is obstruction of the urinary tract, relief of the obstruction (with a [[nephrostomy]] or [[urinary catheter]]) may be necessary. Lack of improvement with fluid resuscitation, therapy-resistant hyperkalemia, metabolic acidosis, or fluid overload may necessitate artificial support in the form of [[dialysis]] or [[hemofiltration]]. Depending on the cause, a proportion of patients will never regain full renal function, thus having [[end stage renal failure]] requiring lifelong [[dialysis]] or a [[kidney transplant]].

==References==

{{Reflist|2}}
{{WH}}
{{WS}}
[[Category:Needs overview]]
[[Category:Medical emergencies]]
[[Category:Kidney diseases]]
[[Category:Organ failure]]
[[Category:Causes of death]]
[[Category:Nephrology]]
[[Category:Emergency medicine]]
[[Category:Intensive care medicine]]

Acute renal failure overview

2020-05-17T19:38:28Z

Ayesha A. Khan: /* Classification */

__NOTOC__
{{Acute renal failure}}
{{CMG}}

==Overview==
Acute renal failure (ARF), also known as acute kidney failure, is a rapid loss of [[renal function]] due to damage to the [[kidney]]s, resulting in retention of nitrogenous ([[urea]] and [[creatinine]]) and non-nitrogenous waste products that are normally excreted by the kidney. Depending on the severity and duration of the renal dysfunction, this accumulation is accompanied by metabolic disturbances, such as [[metabolic acidosis]] (acidification of the blood) and [[hyperkalaemia]] (elevated potassium levels), changes in body [[fluid balance]], and effects on many other organ systems. It can be characterized by [[oliguria]] or [[anuria]] (decrease or cessation of urine production), although ''nonoliguric ARF'' may occur. It is a serious disease and treated as a [[medical emergency]].
==Historical Perspective==
Before the advancement of modern medicine, acute renal failure might be referred to as uremic poisoning. [[Uremia]] was the term used to describe the contamination of the [[blood]] with [[urine]]. Starting around 1847 this term was used to describe reduced urine output, now known as [[oliguria]], which was thought to be caused by the urine's mixing with the blood instead of being voided through the [[urethra]]. Acute renal failure due to [[acute tubular necrosis]] (ATN) was recognised in the 1940s in the United Kingdom, where crush victims during the Battle of Britain developed patchy necrosis of renal tubules, leading to a sudden decrease in renal function.<ref>{{cite journal |author=Bywaters EG, Beall D |title=Crush injuries with impairment of renal function. |journal=[[British Medical Journal|Br Med J]] |volume= |issue=1 |pages=427-32 |year=1941 |pmid=9527411 |doi= |url=http://jasn.asnjournals.org/cgi/pmidlookup?view=long&pmid=9527411}}</ref> During the Korean and Vietnam wars, the incidence of ARF decreased due to better acute management and intravenous infusion of fluids.<ref>{{cite journal |author=Schrier RW, Wang W, Poole B, Mitra A |title=Acute renal failure: definitions, diagnosis, pathogenesis, and therapy |journal=J. Clin. Invest. |volume=114 |issue=1 |pages=5–14 |year=2004 |pmid=15232604 |pmc=437979 |doi=10.1172/JCI22353 |url=}}</ref>
==Classification==
Acute renal failure can complicate a wide spectrum of disorders, and for the purpose of diagnosis and management is divided according to the mechanism that lead to renal compromise. The three categories are pre-renal [[azotemia]] (diseases that cause renal [[hypoperfusion]]), renal azotemia (diseases directly affecting the renal parenchyma), and post-renal azotemia (diseases affecting the [[urinary tract]] causing [[urinary tract obstruction|obstruction]]). However, the first consensus definition described and the most used definition is known as the RIFLE criteria. The acronym combines a classification of 3 levels of renal dysfunction (Risk, Injury, Failure) with 2 clinical outcomes (Loss, ESRD) to give the RIFLE stages of AKI.

==Pathophysiology==
Acute kidney injury (AKI) is the leading cause of nephrology consultation and is associated with high mortality rates. The primary causes of AKI include ischemia, hypoxia or nephrotoxicity. An underlying feature is a rapid decline in GFR usually associated with decreases in renal blood flow. Inflammation represents an important additional component of AKI leading to the extension phase of injury, which may be associated with insensitivity to vasodilator therapy. It is suggested that targeting the extension phase represents an area potential of treatment with the greatest possible impact. The underlying basis of renal injury appears to be impaired energetics of the highly metabolically active nephron segments (i.e., proximal tubules and thick ascending limb) in the renal outer medulla, which can trigger conversion from transient hypoxia to intrinsic renal failure. Injury to kidney cells can be lethal or sublethal. Sublethal injury represents an important component in AKI, as it may profoundly influence GFR and renal blood flow. The nature of the recovery response is mediated by the degree to which sublethal cells can restore normal function and promote regeneration. The successful recovery from AKI depends on the degree to which these repair processes ensue and these may be compromised in elderly or CKD patients. Recent data suggest that AKI represents a potential link to CKD in surviving patients. Finally, earlier diagnosis of AKI represents an important area in treating patients with AKI that has spawned increased awareness of the potential that biomarkers of AKI may play in the future.

==Causes==
[[Glomerular filtration rate]] depends on multiple factors, particularly the [[renal blood flow]]. The pressure difference across the renal vasculature i.e the difference in pressure between the renal arterioles and venules is directly proportional to the [[GFR]]. Any decrease in [[renal blood flow]], injury to the renal tubules or obstruction to the urinary tract outflow can cause decreased urinary output.
==Differentiating Acute renal failure from other Diseases==
Various parameters like [[fractional sodium excretion]], urinary sodium concentration, urine osmolality and U/P [[creatinine]] ratio have been used to diagnose acute renal failure. The [[fractional sodium excretion]] is identifies as the most effective non-invasive test in formulating the differential diagnosis of acute renal failure <ref name="pmid7363517">{{cite journal |author=Espinel CH, Gregory AW |title=Differential diagnosis of acute renal failure |journal=[[Clinical Nephrology]] |volume=13 |issue=2 |pages=73–7 |year=1980 |month=February |pmid=7363517 |doi= |url=}}</ref>.
==Epidemiology and Demographics==
Acute kidney injury is common among hospitalized patients. It affects some 3-7% of patients admitted to the hospital and approximately 25-30% of patients in the [[intensive care unit]] <ref name="isbn1-4160-3110-3">{{cite book |author= |title=Brenner and Rector's The Kidney |publisher=Saunders |location=Philadelphia |year=2007 |pages= |isbn=1-4160-3110-3 |oclc= |doi= |accessdate=}}</ref>.
==Risk Factors==
Acute renal failure always occurs in connection with some other medical condition or event. Being hospitalized with a serious condition requiring intensive care is the biggest risk factor. The risk factors for acute renal failure is divided into three major categories, namely pre-renal factors, renal factors and post-renal factors.
==Diagnosis==
===History and Symptoms===
When a patient presents with [[uremia|uremic]] symptoms, first step is to differentiate an acute from a chronic process. A recent rise in serum [[creatinine]] levels indicate an acute pathology of renal failure, whereas chronically elevated [[creatinine]] levels are seen in [[chronic renal failure]]. However, [[chronic renal insufficiency]] is also associated with [[osteopathy]], [[neuropathy]] and small, scarred [[kidney]]s.
===Laboratory Findings===
Renal failure is generally diagnosed either when [[creatinine]] or [[blood urea nitrogen]] tests are markedly elevated in an ill patient, especially when oliguria is present. Previous measurements of renal function may offer comparison, which is especially important if a patient is known to have [[chronic renal failure]] as well. If the cause is not apparent, a large amount of [[blood test]]s and examination of a [[urine]] specimen is typically performed to elucidate the cause of acute renal failure.
===Ultrasound===
[[Medical ultrasonography]] of the renal tract is essential to rule out obstruction of the urinary tract.
===Other Diagnostic Studies===
Kidney [[biopsy]] may be performed in the setting of acute renal failure, to provide a definitive diagnosis and sometimes an idea of the [[prognosis]], unless the cause is clear and appropriate screening investigations are reassuringly negative.
==Treatment==
===Surgery===
If the cause of the acute renal failure is obstruction of the urinary tract, relief of the obstruction (with a [[nephrostomy]] or [[urinary catheter]]) may be necessary. Lack of improvement with fluid resuscitation, therapy-resistant hyperkalemia, metabolic acidosis, or fluid overload may necessitate artificial support in the form of [[dialysis]] or [[hemofiltration]]. Depending on the cause, a proportion of patients will never regain full renal function, thus having [[end stage renal failure]] requiring lifelong [[dialysis]] or a [[kidney transplant]].

==References==

{{Reflist|2}}
{{WH}}
{{WS}}
[[Category:Needs overview]]
[[Category:Medical emergencies]]
[[Category:Kidney diseases]]
[[Category:Organ failure]]
[[Category:Causes of death]]
[[Category:Nephrology]]
[[Category:Emergency medicine]]
[[Category:Intensive care medicine]]

Septic arthritis overview

2020-05-17T19:09:10Z

Ayesha A. Khan:

__NOTOC__
{{Septic arthritis}}
{{CMG}}; {{AE}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com] {{VSKP}}
==Overview==
Septic arthritis is the one of the most serious medical emergency of a patient present with one or more hot and swollen joints.<ref name="pmid20206778">{{cite journal| author=Mathews CJ, Weston VC, Jones A, Field M, Coakley G| title=Bacterial septic arthritis in adults. | journal=Lancet | year= 2010 | volume= 375 | issue= 9717 | pages= 846-55 | pmid=20206778 | doi=10.1016/S0140-6736(09)61595-6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20206778 }} </ref> It is the most rapidly destructive joint disease.<ref name="pmid9449882">Goldenberg DL (1998) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=9449882 Septic arthritis.] ''Lancet'' 351 (9097):197-202. [http://dx.doi.org/10.1016/S0140-6736(97)09522-6 DOI:10.1016/S0140-6736(97)09522-6] PMID: [https://pubmed.gov/9449882 9449882]</ref> It is most common in patients with longstanding [[rheumatoid arthritis]] and it is a an important consideration in adults presenting with monoarticular arthritis in 80 to 90% of patients. It can involve any joint, but most commonly involves knee > hip > shoulder > ankle.<ref name="pmid3498362">Barton LL, Dunkle LM, Habib FH (1987) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=3498362 Septic arthritis in childhood. A 13-year review.] ''Am J Dis Child'' 141 (8):898-900. PMID: [https://pubmed.gov/3498362 3498362]</ref> Other joints such as [[sacroiliac joint]], [[Sternoclavicular joint|sternoclacicular]] or [[Costoclavicular ligament|costoclavicular joints]] may be involved in patient with history of intravenous drug abuse (IVDA), penetrating trauma, animal or human bites and local [[steroid]] injections. [[Septic arthritis]] is joint [[inflammation]] secondary to an infectious etiology, usually [[bacterial]], but occasionally [[fungal]], [[mycobacterial]], [[viral]], or other uncommon pathogens. [[Septic arthritis]] is usually [[monoarticular]] involving one large joint such as the [[hip]] or [[knee]]; however, [[polyarticular]] [[septic arthritis]] involving multiple or smaller joints may also occur. Though uncommon, [[septic arthritis]] is an [[orthopedic]] emergency that can cause significant joint damage leading to increased [[morbidity]] and [[mortality]].

== Historical Perspective ==
* First case of septic arthritis described in literature by Walter Whitehead in 1902, as "The open method of treating exceptional cases of septic arthritis of the knee".<ref name="pmid20760321">Whitehead W (1902) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=20760321 Observations ON THE "OPEN METHOD" OF TREATING EXCEPTIONAL CASES OF SEPTIC ARTHRITIS OF THE KNEE.] ''Br Med J'' 1 (2164):1523-4. PMID: [https://pubmed.gov/20760321 20760321]</ref>
*An experimental and clinical Study on arthritis deformans described by Nathan PW in 1917.<ref name="pmid19972362">Nathan PW (1917) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=19972362 Arthritis Deformans as an infectious Disease : An experimental and Clinical Study from the Carnegie Laboratory (University and Bellevue Medical College) and the Montefiore Home and Hospital for Chronic Diseases.] ''J Med Res'' 36 (2):187-224.11. PMID: [https://pubmed.gov/19972362 19972362]</ref>
*Surgical management of septic arthritis by By Captain W. Rankin in 1917.<ref name="pmid20768715">Rankin W (1917) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=20768715 ON THE TREATMENT OF CERTAIN SELECTED CASES OF SEPTIC ARTHRITIS OF THE KNEE.] ''Br Med J'' 2 (2957):287-9. PMID: [https://pubmed.gov/20768715 20768715]</ref>

== Classification ==
Septic arthritis broadly classified based on the etiology as [[Gonococcal infection|gonococcal]] or [[Non-gonococcal bacterial arthritis|non-gonococcal]] arthritis and based on the clinical presentation it is classified as mono articular septic arthritis or poly articular septic arthritis.<ref name="pmid12364368">Shirtliff ME, Mader JT (2002) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=12364368 Acute septic arthritis.] ''Clin Microbiol Rev'' 15 (4):527-44. PMID: [https://pubmed.gov/12364368 12364368]</ref><ref name="pmid8412643">Dubost JJ, Fis I, Denis P, Lopitaux R, Soubrier M, Ristori JM et al. (1993) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=8412643 Polyarticular septic arthritis.] ''Medicine (Baltimore)'' 72 (5):296-310. PMID: [https://pubmed.gov/8412643 8412643]</ref>

== Pathophysiology ==
Septic arthritis most commonly develop as a result of hematogenous spreading of bacteria into the [[synovial membrane]], that induces inflammatory reactions. Eventually, release of [[cytokines]] and activation of both host [[Humoral immunity|humoral]] and [[immunological]] response along with bacterial [[virulence factors]] cumulatively damages articular surface and [[cartilage]].<ref name="pmid3288326">Klein RS (1988) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=3288326 Joint infection, with consideration of underlying disease and sources of bacteremia in hematogenous infection.] ''Clin Geriatr Med'' 4 (2):375-94. PMID: [https://pubmed.gov/3288326 3288326]</ref><ref name="pmid737020">Atcheson SG, Ward JR (1978) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=737020 Acute hematogenous osteomyelitis progressing to septic synovitis and eventual pyarthrosis. The vascular pathway.] ''Arthritis Rheum'' 21 (8):968-71. PMID: [https://pubmed.gov/737020 737020]</ref>

==Causes==
Septic arthritis caused by [[bacteria]] or tiny disease-causing [[microorganisms]] that spread through the bloodstream to a synovium. It may also occur when the joint is directly infected with a microorganism from an injury or during surgery. The most common sites for this type of infection are the knee and hip. Most cases of acute septic arthritis are caused by bacteria such as [[staphylococcus]] or [[streptococcus]]. Chronic septic arthritis (which is less common) is caused by organisms such as [[Mycobacterium tuberculosis]] and [[Candida albicans]].

== Differential Diagnosis ==
Patient present with septic arthritis should be differentiate from other causes of acute monoarticular arthritis.
{| border="2" cellpadding="4" cellspacing="0" style="margin: 1em 1em 1em 0; background: #f9f9f9; border: 1px #aaa solid; border-collapse: collapse;" width="75%"
! colspan="5" |Differential diagnosis of acute monoarticular arthritis
|-
! Infectious
! Crystal induced
!Hemorrhagic
!Systemic rheumatological
disorders
!Intra-articular derangement
|-
|valign=top|
* [[Bacterial]] arthritis
* [[Fungal]] arthritis
* [[Mycobacterial]] arthritis
* [[Viral]] arthritis
* [[Lyme disease|Lyme]] arthritis
|valign=top|
* [[Gouty arthritis]] ([[Urate|Urate crystals]])
* [[Pseudogout]] ([[Calcium pyrophosphate deposition disease|Calcium pyrophosphate dihydrate]])
* [[Calcium oxalate]] crystals
|valign=top|
* [[Coagulopathy|Clotting disorders]] (e.g. [[Hemophilia]])
* [[Anti coagulation therapy|Anti-coagulation therapy]]
* [[Fractures|Fracture]]
|valign=top|
* [[Rheumatoid arthritis]]
* [[Spondyloarthritis]]
* [[Systemic lupus erythematosus]]
|valign=top|
* [[Meniscal tear]]
* [[Fractures|Fracture]]
* [[Osteonecrosis]]
|}

== Epidemiology and Demographics ==
* Incidence of septic arthritis approximately varies between 2 to 10 cases per 100,000 per year in the general population.<ref name="pmid8972665">Morgan DS, Fisher D, Merianos A, Currie BJ (1996) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=8972665 An 18 year clinical review of septic arthritis from tropical Australia.] ''Epidemiol Infect'' 117 (3):423-8. PMID: [https://pubmed.gov/8972665 8972665]</ref>
* Incidence of septic arthritis in patients with history of [[rheumatoid arthritis]] and patients with joint prostheses is ~30–70 cases per 100,000 per year.<ref name="pmid8849354">Kaandorp CJ, Van Schaardenburg D, Krijnen P, Habbema JD, van de Laar MA (1995) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=8849354 Risk factors for septic arthritis in patients with joint disease. A prospective study.] ''Arthritis Rheum'' 38 (12):1819-25. PMID: [https://pubmed.gov/8849354 8849354]</ref>
* Incidence of septic arthritis in patients with joint prostheses is 40-68 cases per 100,000 per year.
* The [[Case fatality rate|case-fatality rate]] of septic arthritis is estimated to be 10-25%.<ref name="pmid3883171">Goldenberg DL, Reed JI (1985) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=3883171 Bacterial arthritis.] ''N Engl J Med'' 312 (12):764-71. [http://dx.doi.org/10.1056/NEJM198503213121206 DOI:10.1056/NEJM198503213121206] PMID: [https://pubmed.gov/3883171 3883171]</ref>
* Even after survival from septic arthritis, 25-50% of the patients suffer from irreversible loss of joint function.<ref name="pmid9153550">Kaandorp CJ, Krijnen P, Moens HJ, Habbema JD, van Schaardenburg D (1997) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=9153550 The outcome of bacterial arthritis: a prospective community-based study.] ''Arthritis Rheum'' 40 (5):884-92. [http://dx.doi.org/10.1002/1529-0131(199705)40:5<884::AID-ART15>3.0.CO;2-6 DOI:10.1002/1529-0131(199705)40:5<884::AID-ART15>3.0.CO;2-6] PMID: [https://pubmed.gov/9153550 9153550]</ref><ref name="pmid1882666">Bengtson S, Knutson K (1991) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=1882666 The infected knee arthroplasty. A 6-year follow-up of 357 cases.] ''Acta Orthop Scand'' 62 (4):301-11. PMID: [https://pubmed.gov/1882666 1882666]</ref>

== Risk Factors ==
Most common risk factors that predisposes septic arthritis are [[rheumatoid arthritis]], [[Prosthesis|prosthetic joint]] or [[joint replacement]] and skin infections. Other common risk factors includes:
* Age >80 years
* Recent history of [[bacteremia]]
* Intravenous substance abuse
* [[Corticosteroid]] therapy
* [[Cytotoxic drugs|Cytotoxic chemotherapy]]
* [[Diabetes mellitus]]
* [[Alcoholism]]
* [[Leukemia]]
* [[Granulomatous|Granulomatous diseases]]
* [[Hypogammaglobulinemia]]
* [[Cirrhosis]]
* [[Chronic kidney disease]]

== Natural History, Complications & Prognosis ==
Septic arthritis commonly present with either mono articular involvement associate with [[tenosynovitis]] and [[dermatitis]] ([[Gonococcal infection|gonococcal]]) or polyarticular involvement ([[Non gonococcal arthritis|non gonococcal]]).<ref name="pmid3883171">Goldenberg DL, Reed JI (1985) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=3883171 Bacterial arthritis.] ''N Engl J Med'' 312 (12):764-71. [http://dx.doi.org/10.1056/NEJM198503213121206 DOI:10.1056/NEJM198503213121206] PMID: [https://pubmed.gov/3883171 3883171]</ref> It is more common in patients in extreme age groups with pre existing joint disorders such as [[rheumatoid arthritis]] or predisposing factors such as skin infection.<ref name="pmid1852426">Esterhai JL, Gelb I (1991) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=1852426 Adult septic arthritis.] ''Orthop Clin North Am'' 22 (3):503-14. PMID: [https://pubmed.gov/1852426 1852426]</ref> Prompt diagnosis. rapid initiation of treatment, early physical therapy and mobilization are crucial for the outcome of septic arthritis. Complications of septic arthritis mainly depends on the pre existing joint disease and treatment of current infection. Common complications include [[Degeneration|Joint degeneration]] (~ 40%) [[bacteremia]] (5-20%) [[osteomyelitis]] and [[growth retardation]] (in children)<ref name="pmid5297142">Nelson JD, Koontz WC (1966) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=5297142 Septic arthritis in infants and children: a review of 117 cases.] ''Pediatrics'' 38 (6):966-71. PMID: [https://pubmed.gov/5297142 5297142]</ref> Prognosis of septic arthritis depends on various factors such [[Immune response|host immune response]], pre existing joint disease, presence of risk factors, [[virulence]] of the pathogen and the duration between onset of symptoms and diagnosis.<ref name="pmid769545">Goldenberg DL, Cohen AS (1976) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=769545 Acute infectious arthritis. A review of patients with nongonococcal joint infections (with emphasis on therapy and prognosis).] ''Am J Med'' 60 (3):369-77. PMID: [https://pubmed.gov/769545 769545]</ref>

==Diagnosis==
Patients with history of chronic joint disease and concurrent septic arthritis can be misdiagnosed as acute flareup of underlying chronic disease which often delays the treatment for septic arthritis. So, patients with acute flare of one or two new inflamed joints with underlying chronic joint diseases or with another connective tissue disease, it should be assumed that the joint is septic until proven otherwise, should always rule out concurrent septic arthritis with appropriate diagnostic studies.<ref name="pmid9449882">Goldenberg DL (1998) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=9449882 Septic arthritis.] ''Lancet'' 351 (9097):197-202. [http://dx.doi.org/10.1016/S0140-6736(97)09522-6 DOI:10.1016/S0140-6736(97)09522-6] PMID: [https://pubmed.gov/9449882 9449882]</ref> In patients with acute effusion of unknown etiology, might have concurrent [[Crystal arthropathies|crystal-induced arthritis]] and septic arthritis. So, the synovial fluid should always be cultured and examined for crystals in the evaluation of an acute effusion.<ref name="pmid8823821">{{cite journal| author=Ilahi OA, Swarna U, Hamill RJ, Young EJ, Tullos HS| title=Concomitant crystal and septic arthritis. | journal=Orthopedics | year= 1996 | volume= 19 | issue= 7 | pages= 613-7 | pmid=8823821 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8823821 }} </ref>
===History and Symptoms===
Septic arthritis commonly present with joint pain (knee> hip>shoulder>ankle) associate with [[fever]], [[malaise]] and local joint symptoms such as [[swelling]], [[erythema]] and decreased range of motion at the level of joint. In children, hip is commonly affected. Abrupt onset of a single hot, swollen, and painful joint indicate non gonococcal arthritis.<ref name="pmid3883171">Goldenberg DL, Reed JI (1985) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=3883171 Bacterial arthritis.] ''N Engl J Med'' 312 (12):764-71. [http://dx.doi.org/10.1056/NEJM198503213121206 DOI:10.1056/NEJM198503213121206] PMID: [https://pubmed.gov/3883171 3883171]</ref> It can involve any joint, but most commonly knee is the site of infection in 50% of cases of adults and elderly patients. Hip infection is the most common site in children.<ref name="pmid8972665">Morgan DS, Fisher D, Merianos A, Currie BJ (1996) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=8972665 An 18 year clinical review of septic arthritis from tropical Australia.] ''Epidemiol Infect'' 117 (3):423-8. PMID: [https://pubmed.gov/8972665 8972665]</ref> [[Disseminated gonococcal infection]](DGI) often present initially with migratory [[Polyarthralgia|polyarthralgias]], [[tenosynovitis]], [[dermatitis]], and [[fever]]. Less commonly, patients with DGI will present with purulent joint effusion, most often of the knee or wrist.<ref name="pmid6415361">O'Brien JP, Goldenberg DL, Rice PA (1983) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=6415361 Disseminated gonococcal infection: a prospective analysis of 49 patients and a review of pathophysiology and immune mechanisms.] ''Medicine (Baltimore)'' 62 (6):395-406. PMID: [https://pubmed.gov/6415361 6415361]</ref> Often present with inflamed and tender tendons of the wrist, ankles, and small joints.

=== Physical Examination ===
* [[Swelling]] of the joint that involved
* Decreased range of motion such as pseudo paralysis
* Patient hold the hip in flexed and externally rotated position if SA involving hip.
* If child, unwillingness to bear weight on the affected joint ([[Gait Abnormalities|antalgic gait]])

=== Diagnostic Evaluation ===
{{familytree/start}}
{{familytree| | | | | | | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | | | | | |A01=Hot, swollen joint suspecting septic arthritis}}
{{familytree| | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | |}}
{{familytree| | | | | | | | | | | | | | | | | | | B01 |-|-|-|-|-|-|-|-|-|-|-| B02 | | | | | | | | |B01='''Joint aspiration''' send synovial fluid for [[Gram stain]], culture and cell count|B02='''If dry tap:''' Do image guided joint apiration with [[ultrasound]] or [[CT]]}}
{{familytree| | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | | | | |!| | | | | | | | | |}}
{{familytree| | | | | | | | | | | | | | | | | | | |`|-|-|-|-|-|-|-|-|-|v|-|-|-|'| | | | | | | | | |}}
{{familytree| | | | | | | | | | | | | | | |,|-|-|-|-|-|-|-|-|-|-|-|-|-|^|-|-|-|-|-|-|-|.| | | | | | | | | | |}}
{{familytree| | | | | | | | | | | | | | | D01 | | | | | | | | | | | | | | | | | | | | D02 | | | | | | | | | |D01='''Inflammatory/Purulent''' joint fluid Presence of [[PMN]] 50,000-150,000 cells and mostly [[neutrophils]]|D02='''Non-inflammatory fluid/Crystals''' Suspect non bacterial arthritis}}
{{familytree| | | | | | | |,|-|-|-|-|-|-|-|+|-|-|-|-|-|-|-|.| | | | | | | | | | | | | |!| | | | | | | | | | |}}
{{familytree| | | | | | | E01 | | | | | | E02 | | | | | | E03 | | | | | | | | | | | | |!| | | | | | | | | | |E01='''[[Gram positive cocci]]''' Start empiric [[Vancomycin]] or [[Nafcicillin]]|E02='''[[Gram negative bacilli]]''' Start empiric 3rd generation [[cephalosporins]] + [[aminoglycosides]]|E03='''Negative Gram stain'''}}
{{familytree| | | | | | | |`|-|-|-|v|-|-|-|'| | | | | | | |!| | | | | | | | | | | | | |!| | | | | | |}}
{{familytree| | | | | | | | | | | F01 | | | | | | | | | | |!| | | | | | | | | | | | | |!| | | | | |F01='''Follow-up with synovial fluid culture results'''}}
{{familytree| | | | | | | |,|-|-|-|^|-|-|-|.| | | |,|-|-|-|^|-|-|-|.| | | | | | | | | |!| | | | | | | |}}
{{familytree|boxstyle=text-align: left; | | | | | | | G01 | | | | | | G02 | | | G03 | | | | | | G04 | | | | | | | |!| | | | | | | | | | |G01='''If culture positive''' ❑ Treat for septic arthritis ❑ Change antibiotics according to the culture results ❑ Joint drainage |G02='''If culture negative''' ❑ Assess for true or false positivity of Gram stain ❑ Assess for clinical response|G03='''Immunocompromised''' start empiric [[Vancomycin]] and 3rd generation [[cephalosporins]]|G04='''Immunocompetent''' start empiric [[vancomycin]]}}
{{familytree| | | | | | | | | | | | | | | | | | | |`|-|-|-|-|-|-|-|+|-|-|-|-|-|-|-|-|-|'| | | | | | | | | | |}}
{{familytree| | | | | | | | | | | | | | | | | | | | | | | | | | | I01 | | | | | | | | | | | | | | | |I01='''Wait for culture results'''}}
{{familytree| | | | | | | | | | | | | | | | | | | | | |,|-|-|-|-|-|^|-|-|-|-|.| | | | | | | | | | | |}}
{{familytree|boxstyle=text-align: left; | | | | | | | | | | | | | | | | | | | | | J01 | | | | | | | | | J02 | | | | | | | | | | |J01='''If culture positive''', ❑ Treat for septic arthritis ❑ Change antibiotics according to the culture results ❑ Joint drainage|J02='''If culture negative''' Confirmed non bacterial arthritis and look for alternative diagnosis}}
{{familytree/end}}

== Imaging Studies ==

===X-ray===
X-ray of the joint with septic arthritis are usually normal in the first few days of infection as there is no joint destruction seen usually or may show a preexisting joint disease such as [[rheumatoid arthritis]] or [[osteoarthritis]]. So, the initial x-ray may be useful to determine pre-existing conditions, such as [[osteoarthritis]] or simultaneous [[osteomyelitis]], or may be useful as a baseline image in monitoring the treatment response. However, in the late stages of septic arthritis, X-ray film may show: swelling of the joint capsule and soft tissue around the joint, fat pad displacement, and joint space widening due to localized [[edema]] and effusion.<ref name="pmid7618566">Jaramillo D, Treves ST, Kasser JR, Harper M, Sundel R, Laor T (1995) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=7618566 Osteomyelitis and septic arthritis in children: appropriate use of imaging to guide treatment.] ''AJR Am J Roentgenol'' 165 (2):399-403. [http://dx.doi.org/10.2214/ajr.165.2.7618566 DOI:10.2214/ajr.165.2.7618566] PMID: [https://pubmed.gov/7618566 7618566]</ref>
===CT===
Computerised tomography is used to diagnose ambiguous cases of septic arthritis to differentiate it from other causes of acute arthritis or to determine the extent of bone and soft tissue infections. But, it is less sensitive in the early stages of the disease. In the late stages of septic arthritis, CT shows: visualization of joint effusion, soft tissue swelling, para-articular abscesses, joint space widening due to localized edema, bone erosions, foci of osteitis, and scleroses.<ref name="pmid6725696">Seltzer SE (1984) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=6725696 Value of computed tomography in planning medical and surgical treatment of chronic osteomyelitis.] ''J Comput Assist Tomogr'' 8 (3):482-7. PMID: [https://pubmed.gov/6725696 6725696]</ref><ref name="pmid12364368">Shirtliff ME, Mader JT (2002) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=12364368 Acute septic arthritis.] ''Clin Microbiol Rev'' 15 (4):527-44. PMID: [https://pubmed.gov/12364368 12364368]</ref>

===MRI===
The role of [[MRI]] in the diagnosis of septic arthritis has been increasing in recent years in an effort to detect this entity earlier.<ref name="pmid3714999">Modic MT, Pflanze W, Feiglin DH, Belhobek G (1986) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=3714999 Magnetic resonance imaging of musculoskeletal infections.] ''Radiol Clin North Am'' 24 (2):247-58. PMID: [https://pubmed.gov/3714999 3714999]</ref> Findings are usually evident within 24 hours following the onset of infection and include: [[synovial]] enhancement, perisynovial [[edema]] and joint effusion. Signal abnormalities in the bone marrow can indicate a concomitant [[osteomyelitis]].<ref name="pmid1476623">Tehranzadeh J, Wang F, Mesgarzadeh M (1992) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=1476623 Magnetic resonance imaging of osteomyelitis.] ''Crit Rev Diagn Imaging'' 33 (6):495-534. PMID: [https://pubmed.gov/1476623 1476623]</ref> The [[sensitivity]] and [[specificity]] of MRI for the detection of septic arthritis has been reported to be 100% and 77% respectively.

==Treatment==
===Medical Therapy===
Acute nongonococcal septic arthritis is a medical emergency which causes severe joint destruction and may increase both morbidity and mortality. So prompt diagnosis and treatment with antibiotic therapy and prompt drainage which reduces long-term complications. [[Vancomycin]] is recommended as either empirical therapy for patients with [[Gram-positive cocci]] on a [[synovial fluid]] [[Gram stain]] or as a component of regimen for those with a negative [[Gram stain]] if [[MRSA|methicillin-resistant ''Staphylococcus aureus'' (MRSA)]] is prevalent. If [[Gram-negative bacilli]] are observed, an anti-[[pseudomonal]] [[Cephalosporin]] (e.g., [[Ceftazidime]], [[Cefepime]]) should be administered. [[Carbapenem|Carbapenems]] should be considered in conditions such as colonization or infection by [[ESBL|extended-spectrum β-lactamase]]–producing pathogens. The optimal duration of therapy for septic arthritis remains uncertain. A minimum 3- to 4 week course is suggested for septic arthritis caused by ''[[S. aureus]]'' or [[Gram-negative bacteria]]. The use of [[Corticosteroids]] or intraarticular [[antibiotics]] is not advisable.<ref>{{Cite journal| doi = 10.1016/S0140-6736(09)61595-6| issn = 1474-547X| volume = 375| issue = 9717| pages = 846–855| last1 = Mathews| first1 = Catherine J.| last2 = Weston| first2 = Vivienne C.| last3 = Jones| first3 = Adrian| last4 = Field| first4 = Max| last5 = Coakley| first5 = Gerald| title = Bacterial septic arthritis in adults| journal = Lancet| date = 2010-03-06| pmid = 20206778}}</ref><ref name="pmid23591823">Sharff KA, Richards EP, Townes JM (2013) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=23591823 Clinical management of septic arthritis.] ''Curr Rheumatol Rep'' 15 (6):332. [http://dx.doi.org/10.1007/s11926-013-0332-4 DOI:10.1007/s11926-013-0332-4] PMID: [https://pubmed.gov/23591823 23591823]</ref>
===Surgical Therapy===
Successful treatment of septic arthritis include both anti microbial therapy and removal of intra-articular pus with surgical management. Surgical or arthroscopic management will increase the risk of infections when compared to diagnostic athroscopic procedures without further procedures. Infection rate depends on the type of procedure (open procedures ~17% and arthroscopic procedures 1~1%), duration of the procedure and prior joint disease.<ref name="pmid1637435">Armstrong RW, Bolding F, Joseph R (1992) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=1637435 Septic arthritis following arthroscopy: clinical syndromes and analysis of risk factors.] ''Arthroscopy'' 8 (2):213-23. PMID: [https://pubmed.gov/1637435 1637435]</ref>
Surgical management options include:
* Closed needle aspiration
* Open drainage
* Tidal irrigation
* [[Arthroscopy]]
* Arthrotomy

===Primary Prevention===
Prevention of septic arthritis is possible by intensive treatment of risk factors such as old age patients having [[rheumatoid arthritis]], [[diabetes mellitus]], [[Prostheses|joint prostheses]] or joint surgery and skin infection.<ref name="pmid8849354">Kaandorp CJ, Van Schaardenburg D, Krijnen P, Habbema JD, van de Laar MA (1995) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=8849354 Risk factors for septic arthritis in patients with joint disease. A prospective study.] ''Arthritis Rheum'' 38 (12):1819-25. PMID: [https://pubmed.gov/8849354 8849354]</ref>

==References==
{{reflist|2}}

[[Category:Arthritis]]
[[Category:Medical emergencies]]
[[Category:Disease]]
[[Category:Rheumatology]]

[[Category:Emergency medicine]]

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[Category:Emergency mdicine]]
[[Category:Disease]]
[[Category:Primary care]]
[[Category:Up-To-Date]]
[[Category:Infectious disease]]
[[Category:Rheumatology]]
[[Category:Orthopedics]]

Septic arthritis overview

2020-05-17T19:07:55Z

Ayesha A. Khan: /* Overview */

__NOTOC__
{{Septic arthritis}}
{{CMG}}; {{AE}}{{VSKP}}
==Overview==
Septic arthritis is the one of the most serious medical emergency of a patient present with one or more hot and swollen joints.<ref name="pmid20206778">{{cite journal| author=Mathews CJ, Weston VC, Jones A, Field M, Coakley G| title=Bacterial septic arthritis in adults. | journal=Lancet | year= 2010 | volume= 375 | issue= 9717 | pages= 846-55 | pmid=20206778 | doi=10.1016/S0140-6736(09)61595-6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20206778 }} </ref> It is the most rapidly destructive joint disease.<ref name="pmid9449882">Goldenberg DL (1998) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=9449882 Septic arthritis.] ''Lancet'' 351 (9097):197-202. [http://dx.doi.org/10.1016/S0140-6736(97)09522-6 DOI:10.1016/S0140-6736(97)09522-6] PMID: [https://pubmed.gov/9449882 9449882]</ref> It is most common in patients with longstanding [[rheumatoid arthritis]] and it is a an important consideration in adults presenting with monoarticular arthritis in 80 to 90% of patients. It can involve any joint, but most commonly involves knee > hip > shoulder > ankle.<ref name="pmid3498362">Barton LL, Dunkle LM, Habib FH (1987) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=3498362 Septic arthritis in childhood. A 13-year review.] ''Am J Dis Child'' 141 (8):898-900. PMID: [https://pubmed.gov/3498362 3498362]</ref> Other joints such as [[sacroiliac joint]], [[Sternoclavicular joint|sternoclacicular]] or [[Costoclavicular ligament|costoclavicular joints]] may be involved in patient with history of intravenous drug abuse (IVDA), penetrating trauma, animal or human bites and local [[steroid]] injections. [[Septic arthritis]] is joint [[inflammation]] secondary to an infectious etiology, usually [[bacterial]], but occasionally [[fungal]], [[mycobacterial]], [[viral]], or other uncommon pathogens. [[Septic arthritis]] is usually [[monoarticular]] involving one large joint such as the [[hip]] or [[knee]]; however, [[polyarticular]] [[septic arthritis]] involving multiple or smaller joints may also occur. Though uncommon, [[septic arthritis]] is an [[orthopedic]] emergency that can cause significant joint damage leading to increased [[morbidity]] and [[mortality]].

== Historical Perspective ==
* First case of septic arthritis described in literature by Walter Whitehead in 1902, as "The open method of treating exceptional cases of septic arthritis of the knee".<ref name="pmid20760321">Whitehead W (1902) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=20760321 Observations ON THE "OPEN METHOD" OF TREATING EXCEPTIONAL CASES OF SEPTIC ARTHRITIS OF THE KNEE.] ''Br Med J'' 1 (2164):1523-4. PMID: [https://pubmed.gov/20760321 20760321]</ref>
*An experimental and clinical Study on arthritis deformans described by Nathan PW in 1917.<ref name="pmid19972362">Nathan PW (1917) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=19972362 Arthritis Deformans as an infectious Disease : An experimental and Clinical Study from the Carnegie Laboratory (University and Bellevue Medical College) and the Montefiore Home and Hospital for Chronic Diseases.] ''J Med Res'' 36 (2):187-224.11. PMID: [https://pubmed.gov/19972362 19972362]</ref>
*Surgical management of septic arthritis by By Captain W. Rankin in 1917.<ref name="pmid20768715">Rankin W (1917) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=20768715 ON THE TREATMENT OF CERTAIN SELECTED CASES OF SEPTIC ARTHRITIS OF THE KNEE.] ''Br Med J'' 2 (2957):287-9. PMID: [https://pubmed.gov/20768715 20768715]</ref>

== Classification ==
Septic arthritis broadly classified based on the etiology as [[Gonococcal infection|gonococcal]] or [[Non-gonococcal bacterial arthritis|non-gonococcal]] arthritis and based on the clinical presentation it is classified as mono articular septic arthritis or poly articular septic arthritis.<ref name="pmid12364368">Shirtliff ME, Mader JT (2002) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=12364368 Acute septic arthritis.] ''Clin Microbiol Rev'' 15 (4):527-44. PMID: [https://pubmed.gov/12364368 12364368]</ref><ref name="pmid8412643">Dubost JJ, Fis I, Denis P, Lopitaux R, Soubrier M, Ristori JM et al. (1993) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=8412643 Polyarticular septic arthritis.] ''Medicine (Baltimore)'' 72 (5):296-310. PMID: [https://pubmed.gov/8412643 8412643]</ref>

== Pathophysiology ==
Septic arthritis most commonly develop as a result of hematogenous spreading of bacteria into the [[synovial membrane]], that induces inflammatory reactions. Eventually, release of [[cytokines]] and activation of both host [[Humoral immunity|humoral]] and [[immunological]] response along with bacterial [[virulence factors]] cumulatively damages articular surface and [[cartilage]].<ref name="pmid3288326">Klein RS (1988) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=3288326 Joint infection, with consideration of underlying disease and sources of bacteremia in hematogenous infection.] ''Clin Geriatr Med'' 4 (2):375-94. PMID: [https://pubmed.gov/3288326 3288326]</ref><ref name="pmid737020">Atcheson SG, Ward JR (1978) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=737020 Acute hematogenous osteomyelitis progressing to septic synovitis and eventual pyarthrosis. The vascular pathway.] ''Arthritis Rheum'' 21 (8):968-71. PMID: [https://pubmed.gov/737020 737020]</ref>

==Causes==
Septic arthritis caused by [[bacteria]] or tiny disease-causing [[microorganisms]] that spread through the bloodstream to a synovium. It may also occur when the joint is directly infected with a microorganism from an injury or during surgery. The most common sites for this type of infection are the knee and hip. Most cases of acute septic arthritis are caused by bacteria such as [[staphylococcus]] or [[streptococcus]]. Chronic septic arthritis (which is less common) is caused by organisms such as [[Mycobacterium tuberculosis]] and [[Candida albicans]].

== Differential Diagnosis ==
Patient present with septic arthritis should be differentiate from other causes of acute monoarticular arthritis.
{| border="2" cellpadding="4" cellspacing="0" style="margin: 1em 1em 1em 0; background: #f9f9f9; border: 1px #aaa solid; border-collapse: collapse;" width="75%"
! colspan="5" |Differential diagnosis of acute monoarticular arthritis
|-
! Infectious
! Crystal induced
!Hemorrhagic
!Systemic rheumatological
disorders
!Intra-articular derangement
|-
|valign=top|
* [[Bacterial]] arthritis
* [[Fungal]] arthritis
* [[Mycobacterial]] arthritis
* [[Viral]] arthritis
* [[Lyme disease|Lyme]] arthritis
|valign=top|
* [[Gouty arthritis]] ([[Urate|Urate crystals]])
* [[Pseudogout]] ([[Calcium pyrophosphate deposition disease|Calcium pyrophosphate dihydrate]])
* [[Calcium oxalate]] crystals
|valign=top|
* [[Coagulopathy|Clotting disorders]] (e.g. [[Hemophilia]])
* [[Anti coagulation therapy|Anti-coagulation therapy]]
* [[Fractures|Fracture]]
|valign=top|
* [[Rheumatoid arthritis]]
* [[Spondyloarthritis]]
* [[Systemic lupus erythematosus]]
|valign=top|
* [[Meniscal tear]]
* [[Fractures|Fracture]]
* [[Osteonecrosis]]
|}

== Epidemiology and Demographics ==
* Incidence of septic arthritis approximately varies between 2 to 10 cases per 100,000 per year in the general population.<ref name="pmid8972665">Morgan DS, Fisher D, Merianos A, Currie BJ (1996) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=8972665 An 18 year clinical review of septic arthritis from tropical Australia.] ''Epidemiol Infect'' 117 (3):423-8. PMID: [https://pubmed.gov/8972665 8972665]</ref>
* Incidence of septic arthritis in patients with history of [[rheumatoid arthritis]] and patients with joint prostheses is ~30–70 cases per 100,000 per year.<ref name="pmid8849354">Kaandorp CJ, Van Schaardenburg D, Krijnen P, Habbema JD, van de Laar MA (1995) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=8849354 Risk factors for septic arthritis in patients with joint disease. A prospective study.] ''Arthritis Rheum'' 38 (12):1819-25. PMID: [https://pubmed.gov/8849354 8849354]</ref>
* Incidence of septic arthritis in patients with joint prostheses is 40-68 cases per 100,000 per year.
* The [[Case fatality rate|case-fatality rate]] of septic arthritis is estimated to be 10-25%.<ref name="pmid3883171">Goldenberg DL, Reed JI (1985) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=3883171 Bacterial arthritis.] ''N Engl J Med'' 312 (12):764-71. [http://dx.doi.org/10.1056/NEJM198503213121206 DOI:10.1056/NEJM198503213121206] PMID: [https://pubmed.gov/3883171 3883171]</ref>
* Even after survival from septic arthritis, 25-50% of the patients suffer from irreversible loss of joint function.<ref name="pmid9153550">Kaandorp CJ, Krijnen P, Moens HJ, Habbema JD, van Schaardenburg D (1997) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=9153550 The outcome of bacterial arthritis: a prospective community-based study.] ''Arthritis Rheum'' 40 (5):884-92. [http://dx.doi.org/10.1002/1529-0131(199705)40:5<884::AID-ART15>3.0.CO;2-6 DOI:10.1002/1529-0131(199705)40:5<884::AID-ART15>3.0.CO;2-6] PMID: [https://pubmed.gov/9153550 9153550]</ref><ref name="pmid1882666">Bengtson S, Knutson K (1991) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=1882666 The infected knee arthroplasty. A 6-year follow-up of 357 cases.] ''Acta Orthop Scand'' 62 (4):301-11. PMID: [https://pubmed.gov/1882666 1882666]</ref>

== Risk Factors ==
Most common risk factors that predisposes septic arthritis are [[rheumatoid arthritis]], [[Prosthesis|prosthetic joint]] or [[joint replacement]] and skin infections. Other common risk factors includes:
* Age >80 years
* Recent history of [[bacteremia]]
* Intravenous substance abuse
* [[Corticosteroid]] therapy
* [[Cytotoxic drugs|Cytotoxic chemotherapy]]
* [[Diabetes mellitus]]
* [[Alcoholism]]
* [[Leukemia]]
* [[Granulomatous|Granulomatous diseases]]
* [[Hypogammaglobulinemia]]
* [[Cirrhosis]]
* [[Chronic kidney disease]]

== Natural History, Complications & Prognosis ==
Septic arthritis commonly present with either mono articular involvement associate with [[tenosynovitis]] and [[dermatitis]] ([[Gonococcal infection|gonococcal]]) or polyarticular involvement ([[Non gonococcal arthritis|non gonococcal]]).<ref name="pmid3883171">Goldenberg DL, Reed JI (1985) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=3883171 Bacterial arthritis.] ''N Engl J Med'' 312 (12):764-71. [http://dx.doi.org/10.1056/NEJM198503213121206 DOI:10.1056/NEJM198503213121206] PMID: [https://pubmed.gov/3883171 3883171]</ref> It is more common in patients in extreme age groups with pre existing joint disorders such as [[rheumatoid arthritis]] or predisposing factors such as skin infection.<ref name="pmid1852426">Esterhai JL, Gelb I (1991) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=1852426 Adult septic arthritis.] ''Orthop Clin North Am'' 22 (3):503-14. PMID: [https://pubmed.gov/1852426 1852426]</ref> Prompt diagnosis. rapid initiation of treatment, early physical therapy and mobilization are crucial for the outcome of septic arthritis. Complications of septic arthritis mainly depends on the pre existing joint disease and treatment of current infection. Common complications include [[Degeneration|Joint degeneration]] (~ 40%) [[bacteremia]] (5-20%) [[osteomyelitis]] and [[growth retardation]] (in children)<ref name="pmid5297142">Nelson JD, Koontz WC (1966) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=5297142 Septic arthritis in infants and children: a review of 117 cases.] ''Pediatrics'' 38 (6):966-71. PMID: [https://pubmed.gov/5297142 5297142]</ref> Prognosis of septic arthritis depends on various factors such [[Immune response|host immune response]], pre existing joint disease, presence of risk factors, [[virulence]] of the pathogen and the duration between onset of symptoms and diagnosis.<ref name="pmid769545">Goldenberg DL, Cohen AS (1976) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=769545 Acute infectious arthritis. A review of patients with nongonococcal joint infections (with emphasis on therapy and prognosis).] ''Am J Med'' 60 (3):369-77. PMID: [https://pubmed.gov/769545 769545]</ref>

==Diagnosis==
Patients with history of chronic joint disease and concurrent septic arthritis can be misdiagnosed as acute flareup of underlying chronic disease which often delays the treatment for septic arthritis. So, patients with acute flare of one or two new inflamed joints with underlying chronic joint diseases or with another connective tissue disease, it should be assumed that the joint is septic until proven otherwise, should always rule out concurrent septic arthritis with appropriate diagnostic studies.<ref name="pmid9449882">Goldenberg DL (1998) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=9449882 Septic arthritis.] ''Lancet'' 351 (9097):197-202. [http://dx.doi.org/10.1016/S0140-6736(97)09522-6 DOI:10.1016/S0140-6736(97)09522-6] PMID: [https://pubmed.gov/9449882 9449882]</ref> In patients with acute effusion of unknown etiology, might have concurrent [[Crystal arthropathies|crystal-induced arthritis]] and septic arthritis. So, the synovial fluid should always be cultured and examined for crystals in the evaluation of an acute effusion.<ref name="pmid8823821">{{cite journal| author=Ilahi OA, Swarna U, Hamill RJ, Young EJ, Tullos HS| title=Concomitant crystal and septic arthritis. | journal=Orthopedics | year= 1996 | volume= 19 | issue= 7 | pages= 613-7 | pmid=8823821 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8823821 }} </ref>
===History and Symptoms===
Septic arthritis commonly present with joint pain (knee> hip>shoulder>ankle) associate with [[fever]], [[malaise]] and local joint symptoms such as [[swelling]], [[erythema]] and decreased range of motion at the level of joint. In children, hip is commonly affected. Abrupt onset of a single hot, swollen, and painful joint indicate non gonococcal arthritis.<ref name="pmid3883171">Goldenberg DL, Reed JI (1985) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=3883171 Bacterial arthritis.] ''N Engl J Med'' 312 (12):764-71. [http://dx.doi.org/10.1056/NEJM198503213121206 DOI:10.1056/NEJM198503213121206] PMID: [https://pubmed.gov/3883171 3883171]</ref> It can involve any joint, but most commonly knee is the site of infection in 50% of cases of adults and elderly patients. Hip infection is the most common site in children.<ref name="pmid8972665">Morgan DS, Fisher D, Merianos A, Currie BJ (1996) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=8972665 An 18 year clinical review of septic arthritis from tropical Australia.] ''Epidemiol Infect'' 117 (3):423-8. PMID: [https://pubmed.gov/8972665 8972665]</ref> [[Disseminated gonococcal infection]](DGI) often present initially with migratory [[Polyarthralgia|polyarthralgias]], [[tenosynovitis]], [[dermatitis]], and [[fever]]. Less commonly, patients with DGI will present with purulent joint effusion, most often of the knee or wrist.<ref name="pmid6415361">O'Brien JP, Goldenberg DL, Rice PA (1983) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=6415361 Disseminated gonococcal infection: a prospective analysis of 49 patients and a review of pathophysiology and immune mechanisms.] ''Medicine (Baltimore)'' 62 (6):395-406. PMID: [https://pubmed.gov/6415361 6415361]</ref> Often present with inflamed and tender tendons of the wrist, ankles, and small joints.

=== Physical Examination ===
* [[Swelling]] of the joint that involved
* Decreased range of motion such as pseudo paralysis
* Patient hold the hip in flexed and externally rotated position if SA involving hip.
* If child, unwillingness to bear weight on the affected joint ([[Gait Abnormalities|antalgic gait]])

=== Diagnostic Evaluation ===
{{familytree/start}}
{{familytree| | | | | | | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | | | | | |A01=Hot, swollen joint suspecting septic arthritis}}
{{familytree| | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | |}}
{{familytree| | | | | | | | | | | | | | | | | | | B01 |-|-|-|-|-|-|-|-|-|-|-| B02 | | | | | | | | |B01='''Joint aspiration''' send synovial fluid for [[Gram stain]], culture and cell count|B02='''If dry tap:''' Do image guided joint apiration with [[ultrasound]] or [[CT]]}}
{{familytree| | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | | | | |!| | | | | | | | | |}}
{{familytree| | | | | | | | | | | | | | | | | | | |`|-|-|-|-|-|-|-|-|-|v|-|-|-|'| | | | | | | | | |}}
{{familytree| | | | | | | | | | | | | | | |,|-|-|-|-|-|-|-|-|-|-|-|-|-|^|-|-|-|-|-|-|-|.| | | | | | | | | | |}}
{{familytree| | | | | | | | | | | | | | | D01 | | | | | | | | | | | | | | | | | | | | D02 | | | | | | | | | |D01='''Inflammatory/Purulent''' joint fluid Presence of [[PMN]] 50,000-150,000 cells and mostly [[neutrophils]]|D02='''Non-inflammatory fluid/Crystals''' Suspect non bacterial arthritis}}
{{familytree| | | | | | | |,|-|-|-|-|-|-|-|+|-|-|-|-|-|-|-|.| | | | | | | | | | | | | |!| | | | | | | | | | |}}
{{familytree| | | | | | | E01 | | | | | | E02 | | | | | | E03 | | | | | | | | | | | | |!| | | | | | | | | | |E01='''[[Gram positive cocci]]''' Start empiric [[Vancomycin]] or [[Nafcicillin]]|E02='''[[Gram negative bacilli]]''' Start empiric 3rd generation [[cephalosporins]] + [[aminoglycosides]]|E03='''Negative Gram stain'''}}
{{familytree| | | | | | | |`|-|-|-|v|-|-|-|'| | | | | | | |!| | | | | | | | | | | | | |!| | | | | | |}}
{{familytree| | | | | | | | | | | F01 | | | | | | | | | | |!| | | | | | | | | | | | | |!| | | | | |F01='''Follow-up with synovial fluid culture results'''}}
{{familytree| | | | | | | |,|-|-|-|^|-|-|-|.| | | |,|-|-|-|^|-|-|-|.| | | | | | | | | |!| | | | | | | |}}
{{familytree|boxstyle=text-align: left; | | | | | | | G01 | | | | | | G02 | | | G03 | | | | | | G04 | | | | | | | |!| | | | | | | | | | |G01='''If culture positive''' ❑ Treat for septic arthritis ❑ Change antibiotics according to the culture results ❑ Joint drainage |G02='''If culture negative''' ❑ Assess for true or false positivity of Gram stain ❑ Assess for clinical response|G03='''Immunocompromised''' start empiric [[Vancomycin]] and 3rd generation [[cephalosporins]]|G04='''Immunocompetent''' start empiric [[vancomycin]]}}
{{familytree| | | | | | | | | | | | | | | | | | | |`|-|-|-|-|-|-|-|+|-|-|-|-|-|-|-|-|-|'| | | | | | | | | | |}}
{{familytree| | | | | | | | | | | | | | | | | | | | | | | | | | | I01 | | | | | | | | | | | | | | | |I01='''Wait for culture results'''}}
{{familytree| | | | | | | | | | | | | | | | | | | | | |,|-|-|-|-|-|^|-|-|-|-|.| | | | | | | | | | | |}}
{{familytree|boxstyle=text-align: left; | | | | | | | | | | | | | | | | | | | | | J01 | | | | | | | | | J02 | | | | | | | | | | |J01='''If culture positive''', ❑ Treat for septic arthritis ❑ Change antibiotics according to the culture results ❑ Joint drainage|J02='''If culture negative''' Confirmed non bacterial arthritis and look for alternative diagnosis}}
{{familytree/end}}

== Imaging Studies ==

===X-ray===
X-ray of the joint with septic arthritis are usually normal in the first few days of infection as there is no joint destruction seen usually or may show a preexisting joint disease such as [[rheumatoid arthritis]] or [[osteoarthritis]]. So, the initial x-ray may be useful to determine pre-existing conditions, such as [[osteoarthritis]] or simultaneous [[osteomyelitis]], or may be useful as a baseline image in monitoring the treatment response. However, in the late stages of septic arthritis, X-ray film may show: swelling of the joint capsule and soft tissue around the joint, fat pad displacement, and joint space widening due to localized [[edema]] and effusion.<ref name="pmid7618566">Jaramillo D, Treves ST, Kasser JR, Harper M, Sundel R, Laor T (1995) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=7618566 Osteomyelitis and septic arthritis in children: appropriate use of imaging to guide treatment.] ''AJR Am J Roentgenol'' 165 (2):399-403. [http://dx.doi.org/10.2214/ajr.165.2.7618566 DOI:10.2214/ajr.165.2.7618566] PMID: [https://pubmed.gov/7618566 7618566]</ref>
===CT===
Computerised tomography is used to diagnose ambiguous cases of septic arthritis to differentiate it from other causes of acute arthritis or to determine the extent of bone and soft tissue infections. But, it is less sensitive in the early stages of the disease. In the late stages of septic arthritis, CT shows: visualization of joint effusion, soft tissue swelling, para-articular abscesses, joint space widening due to localized edema, bone erosions, foci of osteitis, and scleroses.<ref name="pmid6725696">Seltzer SE (1984) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=6725696 Value of computed tomography in planning medical and surgical treatment of chronic osteomyelitis.] ''J Comput Assist Tomogr'' 8 (3):482-7. PMID: [https://pubmed.gov/6725696 6725696]</ref><ref name="pmid12364368">Shirtliff ME, Mader JT (2002) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=12364368 Acute septic arthritis.] ''Clin Microbiol Rev'' 15 (4):527-44. PMID: [https://pubmed.gov/12364368 12364368]</ref>

===MRI===
The role of [[MRI]] in the diagnosis of septic arthritis has been increasing in recent years in an effort to detect this entity earlier.<ref name="pmid3714999">Modic MT, Pflanze W, Feiglin DH, Belhobek G (1986) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=3714999 Magnetic resonance imaging of musculoskeletal infections.] ''Radiol Clin North Am'' 24 (2):247-58. PMID: [https://pubmed.gov/3714999 3714999]</ref> Findings are usually evident within 24 hours following the onset of infection and include: [[synovial]] enhancement, perisynovial [[edema]] and joint effusion. Signal abnormalities in the bone marrow can indicate a concomitant [[osteomyelitis]].<ref name="pmid1476623">Tehranzadeh J, Wang F, Mesgarzadeh M (1992) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=1476623 Magnetic resonance imaging of osteomyelitis.] ''Crit Rev Diagn Imaging'' 33 (6):495-534. PMID: [https://pubmed.gov/1476623 1476623]</ref> The [[sensitivity]] and [[specificity]] of MRI for the detection of septic arthritis has been reported to be 100% and 77% respectively.

==Treatment==
===Medical Therapy===
Acute nongonococcal septic arthritis is a medical emergency which causes severe joint destruction and may increase both morbidity and mortality. So prompt diagnosis and treatment with antibiotic therapy and prompt drainage which reduces long-term complications. [[Vancomycin]] is recommended as either empirical therapy for patients with [[Gram-positive cocci]] on a [[synovial fluid]] [[Gram stain]] or as a component of regimen for those with a negative [[Gram stain]] if [[MRSA|methicillin-resistant ''Staphylococcus aureus'' (MRSA)]] is prevalent. If [[Gram-negative bacilli]] are observed, an anti-[[pseudomonal]] [[Cephalosporin]] (e.g., [[Ceftazidime]], [[Cefepime]]) should be administered. [[Carbapenem|Carbapenems]] should be considered in conditions such as colonization or infection by [[ESBL|extended-spectrum β-lactamase]]–producing pathogens. The optimal duration of therapy for septic arthritis remains uncertain. A minimum 3- to 4 week course is suggested for septic arthritis caused by ''[[S. aureus]]'' or [[Gram-negative bacteria]]. The use of [[Corticosteroids]] or intraarticular [[antibiotics]] is not advisable.<ref>{{Cite journal| doi = 10.1016/S0140-6736(09)61595-6| issn = 1474-547X| volume = 375| issue = 9717| pages = 846–855| last1 = Mathews| first1 = Catherine J.| last2 = Weston| first2 = Vivienne C.| last3 = Jones| first3 = Adrian| last4 = Field| first4 = Max| last5 = Coakley| first5 = Gerald| title = Bacterial septic arthritis in adults| journal = Lancet| date = 2010-03-06| pmid = 20206778}}</ref><ref name="pmid23591823">Sharff KA, Richards EP, Townes JM (2013) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=23591823 Clinical management of septic arthritis.] ''Curr Rheumatol Rep'' 15 (6):332. [http://dx.doi.org/10.1007/s11926-013-0332-4 DOI:10.1007/s11926-013-0332-4] PMID: [https://pubmed.gov/23591823 23591823]</ref>
===Surgical Therapy===
Successful treatment of septic arthritis include both anti microbial therapy and removal of intra-articular pus with surgical management. Surgical or arthroscopic management will increase the risk of infections when compared to diagnostic athroscopic procedures without further procedures. Infection rate depends on the type of procedure (open procedures ~17% and arthroscopic procedures 1~1%), duration of the procedure and prior joint disease.<ref name="pmid1637435">Armstrong RW, Bolding F, Joseph R (1992) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=1637435 Septic arthritis following arthroscopy: clinical syndromes and analysis of risk factors.] ''Arthroscopy'' 8 (2):213-23. PMID: [https://pubmed.gov/1637435 1637435]</ref>
Surgical management options include:
* Closed needle aspiration
* Open drainage
* Tidal irrigation
* [[Arthroscopy]]
* Arthrotomy

===Primary Prevention===
Prevention of septic arthritis is possible by intensive treatment of risk factors such as old age patients having [[rheumatoid arthritis]], [[diabetes mellitus]], [[Prostheses|joint prostheses]] or joint surgery and skin infection.<ref name="pmid8849354">Kaandorp CJ, Van Schaardenburg D, Krijnen P, Habbema JD, van de Laar MA (1995) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=8849354 Risk factors for septic arthritis in patients with joint disease. A prospective study.] ''Arthritis Rheum'' 38 (12):1819-25. PMID: [https://pubmed.gov/8849354 8849354]</ref>

==References==
{{reflist|2}}

[[Category:Arthritis]]
[[Category:Medical emergencies]]
[[Category:Disease]]
[[Category:Rheumatology]]

[[Category:Emergency medicine]]

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[Category:Emergency mdicine]]
[[Category:Disease]]
[[Category:Primary care]]
[[Category:Up-To-Date]]
[[Category:Infectious disease]]
[[Category:Rheumatology]]
[[Category:Orthopedics]]

Osteoarthritis overview

2020-05-13T16:25:51Z

Ayesha A. Khan: /* Pathophysiology */

[[File:TITLE.jpg|thumb|400x400px|'''Osteoarthritis of the Medial Side of the Knee.''']]
<div style="-webkit-user-select: none;">
{| class="infobox" style="position: fixed; top: 65%; right: 10px; margin: 0 0 0 0; border: 0; float: right;"
|-
| {{#ev:youtube|https://https://www.youtube.com/watch?v=sUOlmI-naFs|350}}
|-
|}
__NOTOC__
{{Osteoarthritis}}
{{CMG}}; {{AE}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com] [[User:DrMars|Mohammadmain Rezazadehsaatlou]]'''''<nowiki/>''''', [[User:Irfan Dotani|Irfan Dotani]]

=='''Overview'''==
'''Osteoarthritis / Osteoarthrosis''' (OA, also known as [[degenerative joint disease]], [[degenerative arthritis]], arthrosis or in more colloquial terms "wear and tear") is the most common form of arthritis, caused by wearing of the [[cartilage]] that covers and cushions joint spaces. As the cartilage wears away, the patient may experience pain described as "weight-bearing" whenever walking and standing. Due to the movement limitations caused by pain, regional muscles may experience [[atrophy]]. [[Ligament|Ligaments]] may become laxer as well due to this. OA is derived from the Greek word "''osteo''", meaning "of the bone", "''arthro''", meaning "joint", and "''itis''", meaning [[inflammation]], although inflammation is not a common finding in this regard. [[Osteoarthritis|OA]] possesses a great degree of variability in disease onset, progression, and severity. [[Osteoarthritis|OA]] is characterized by a variety of structural and functional impairments occurring in an involved joint. Destruction, degeneration, articular cartilage loss, and even the soft tissue involvement are the main pathological process of this disease. It can be diagnosed through radiographic evaluations. Moreover, clinical sign and symptoms are helpful in the final diagnosis of this disease. OA can be defined radiologically, clinically, or pathologically, with radiographic OA being considered as the reference standard. The symptoms that are consistently associated with OA are joint pain, stiffness, swelling, and limitation of joint function. Few individuals who present these symptoms may not demonstrate radiographic OA. However, others confirmed to have OA using radiographic techniques may not present with clinical manifestations of the disease. These unique characteristics have made it difficult to identify the underlying mechanisms contributing to the disease as well as the treatments for reducing the incidence and severity of the disease. In addition, the stimuli that may initiate the processes associated with OA are multifactorial and include occupational and non-occupational (e.g., genetics, obesity, age, etc.) factors. [[Osteoarthritis|OA]] affects nearly 43 million patients in [[United States]] and almost 15% of the world population, accounting for 25% of visits to [[Primary care physician|primary care physicians]], and half of all [[NSAID]] (Non-Steroidal Anti-Inflammatory Drugs) [[Medical prescription|prescriptions]]. It is estimated that 80% of the population will have [[Radiograph|radiographic]] evidence of OA by age 65, although only 60% of those will be There is no recent discovery of a cure for OA, as cartilage has not been induced to regenerate. However, if OA is caused by cartilage damage (for example as a result of an injury) Autologous Chondrocyte Implantation may be a possible treatment. Other treatments are with NSAIDs, local injections of [[glucocorticoid]] or [[hyaluronan]], and in severe cases, with [[joint replacement]] surgery. Many physicians have also reported good pain relief by treating ligaments (which is responsible for a bone to bone connection) with [[Prolotherapy]]. Clinical trials employing [[Tissue engineering|tissue-engineering]] methods have demonstrated regeneration of cartilage in damaged knees, including those that have progressed to osteoarthritis. Furthermore, in January 2007, Johns Hopkins University was offering to license a technology of this kind, listing several clinical competitors in its market analysis. Osteoarthritis is capable of influencing any joint in the human body; meanwhile, the most commonly affected joints are the knee and hip joints given that the degree of weight bearing required of these joints is immense. Other joints, such as the [[distal interphalangeal joints]] of the fingers and shoulder joints are also commonly affected as well. The economic burden of OA for United States economy is more than $60 billion per year; which has more economic pressure than [[rheumatoid arthritis]]. This cost can be considered into two subgroups: the medical related costs and the lost expediency of patients at work <ref name="pmid25586654">{{cite journal |vauthors=Peter WF, Dekker J, Tilbury C, Tordoir RL, Verdegaal SH, Onstenk R, Bénard MR, Vehmeijer SB, Fiocco M, Vermeulen HM, van der Linden-van der Zwaag HM, Nelissen RG, Vliet Vlieland TP |title=The association between comorbidities and pain, physical function and quality of life following hip and knee arthroplasty |journal=Rheumatol. Int. |volume=35 |issue=7 |pages=1233–41 |date=July 2015 |pmid=25586654 |pmc=4436688 |doi=10.1007/s00296-015-3211-7 |url=}}</ref>

=='''Historical Perspective'''==

The earliest descriptions of [[Osteoarthritis|OA]] were provided by Heberden and Haygarth in the 19th century. In the 1930s and 1940s, Dr. Stecher showed that there were two forms of OA: idiopathic and post-traumatic. [[Osteoarthritis overview#cite note-5|[5]]] In the 1950s, the connection between Heberden’s nodes and large joint OA were revealed by Kellgren and Moore. In this regard, the first x-ray grading system for OA was developed by Jonas Kellgren and John Lawrence in the 1950s. Surgical management of OA was developed in the 1960s by Drs. Charnley and McKee <ref name="pmid22632687">{{cite journal |vauthors=Suri P, Morgenroth DC, Hunter DJ |title=Epidemiology of osteoarthritis and associated comorbidities |journal=PM R |volume=4 |issue=5 Suppl |pages=S10–9 |date=May 2012 |pmid=22632687 |doi=10.1016/j.pmrj.2012.01.007 |url=}}</ref>

=='''Classification'''==

Osteoarthritis is radiographically classified depending on the degree of joint involvement. The Kellgren-Lawrence is a common method to classify the severity of OA in the knee using five different grades. This classification was proposed by Kellgren et al. in 1957 and was then accepted by WHO in 1961 <ref name="pmid25628884">{{cite journal |vauthors=Hardcastle SA, Dieppe P, Gregson CL, Davey Smith G, Tobias JH |title=Osteoarthritis and bone mineral density: are strong bones bad for joints? |journal=Bonekey Rep |volume=4 |issue= |pages=624 |date=2015 |pmid=25628884 |pmc=4303262 |doi=10.1038/bonekey.2014.119 |url=}}</ref>...

==Pathophysiology==
[[Osteoarthritis]] (OA) is a well-known [[degenerative joint disease]] influencing millions of people worldwide. [[Osteoarthritis]] is a complex disease caused by changes in the tissues' [[homeostasis]] of articular [[cartilages]] and [[subchondral bones]]. The [[cell/extracellular matrix]] (ECM) and their interactions play an important role in the [[pathophysiology]] of articular [[cartilage]] and the occurrence of [[Osteoarthritis]]. Consequently, the main feature of [[OA]] is that after this process is involved, the [[articular]] [[cartilages]] of the involved joint no longer will have a normal acting system because the destruction of the articular [[cartilage]]s can no longer act as [[shock]] absorber. abnormal [[integrin]] expression alters cell/ECM signaling and modifies [[chondrocyte]] synthesis, with the following imbalance of [[destructive]] cytokines over regulatory factors. [[IL-1]], [[TNF-alpha]] and other [[pro-catabolic]] [[cytokines]] activate the enzymatic degradation of [[cartilage]] matrix and are not counterbalanced by adequate synthesis of inhibitors. The main [[enzymes]] involved in [[ECM]] breakdown are [[metalloproteinases]] (MMPs), which are sequentially activated by an amplifying cascade. [[MMP]] activity is partially inhibited by the tissue inhibitors of MMPs (TIMPs), whose synthesis is low compared with MMP production in OA [[cartilage]].

==Causes==
Osteoarthritis (OA) is a result of a variety of disorders that lead to structural and functional failure in involved joints. Osteoarthritis, traditionally, has been considered as a disease specifically related to articular cartilage. Presently, it's been proven that OA influencing the whole joint system consisted of capsule, and synovium, subchondral bone, cartilage, menisci, ligaments, and periarticular muscle.

==Differentiating {{PAGENAME}} from Other Diseases==
OA must be differentiated from other diseases that cause joint impairment and other related signs and symptoms such as [[Rheumatoid arthritis]], [[Gout]], Joint [[tuberculosis]].

==Epidemiology and Demographics==
OA is one of the most frequent diagnoses and is the leading cause of disability among the adult population in the USA. According to the National Health and Nutrition Examination Survey (NHANES), more than 26 million people in the USA were diagnosed with different forms of OA. The National Health Interview Survey (NHIS) reported that the 46.4 million Americans and 21.6% of Americans adults were diagnosed with arthritis. OA can involve any joint, but knees, hips, hands, are most common sites for this involvement.

==Risk Factors==
Osteoarthritis is a multifactorial disease and the interactions between systemic and local factors play important role in development and prognosis of OA.

==Screening==
Routine screening for osteoarthritis is not indicated unless the patient is symptomatic.

==Natural History, Complications, and Prognosis==
===Natural History===
The orthopedic surgeons are frequently asked by their patients regarding the final outcome and the prognosis of their diseased joint/joints caused by OA. Information about the natural history of OA is very important for upcoming determinations and planning for patients management. A small number of studies are available studied the role of the radiographic findings, joint congruence, or even the daily life activity on the OA progression.

===Complications===
OA is a leading cause of morbidity having significant effects on patients life and the health care system and even it could cause heavy economic burden. According to the American Academy of Orthopedic Surgeons report movement limitation are found in 80% of adults diagnosed with osteoarthritis. Meanwhile, 25% of these patients facing difficulties in their of daily living activities. 11% of them need personal care assistance and 14% required help with their routine needs.

===Prognosis===
Most osteoarthritis cases do stabilize. Some osteoarthritis cases progress. A small number of osteoarthritis patients improve spontaneously.

==Diagnosis==
===Diagnostic Criteria===
There is no specific signs and symptoms of osteoarthritis (OA) diagnosis. Although, osteoarthritis may be diagnosed based on several elements such as patients age, past medical history, and available symptoms. Osteoarthritis of the major joints is diagnosed using a combination of patients past medical history, physical examination, and variety of lab tests including imaging studies such as X-ray.

===History and Symptoms===
Patients' past medical history is the most useful tool for the osteoarthritis diagnosis.

===Physical Examination===
A physical examination following the medical history is necessary for medical doctors to reach an exact diagnosis. In OA, loss or limited range of motion in specific joints, swelling, tenderness, and bony growths in the surrounding area are the most important keys in physical examination of OA cases.

===Laboratory Findings===
If the OA diagnosis is in doubt, laboratory tests are used to help doctors get a confirmation regarding a suspected diagnosis of osteoarthritis.

===Imaging Findings===
X-Ray and CT-Scan are the most common tools used in this regard.

===Other Diagnostic Studies===

==Treatment==
===Medical Therapy===
Nonpharmacologic therapy is consisted of physical therapy and specific type of physical exercises, bracing and splinting. Physical therapy results in short-term pain reduction, and improvement in physical function in the diseased joint to preserve its the ability for daily tasks like walking, dressing, and even bathing. Having moderate activity strengthens the muscles around the diseased joint and this reduces stress and increases the stability of the joint system. On the other hand, resting is another important healing factor in OA. Bracing and splinting as other methods help to support painful or unstable joints. Using a cane can help decrease the weight pressure in diseased hip or knee, but it should be used on the contralateral side of the affected joint.

===Surgery===
Surgical interventions in OA cases should be considered when the symptoms have no response to the first line treatments because osteoarthritis symptoms can be successfully managed through [[Osteoarthritis medical therapy|non-surgical]] care. For some, however, if they are experiencing severe joint damage, extreme pain, or very restricted mobility, surgery may be a viable option in this regard. The main indication criteria for surgery in OA is pain and disabilities despite the medical treatments. The most common and effective surgical intervention are arthroscopic surgery, osteotomy, and arthroplasty (total joint replacement). Considering the potential benefits of surgery like pain relief, improved movement, increased patients status, and actual disease prognosis, it should be remembered that any surgical interventions have risks. Meanwhile, overweight patients or patients with co-morbidities have higher risk of operation. The current joint prostheses have an expected functional usage for almost 15 to 20 years.

===Prevention===

Primary prevention for OA include :
* [[Weight loss]]
* Physical activity
* Injury prevention
* Control infectious disease
* Avoidance of [[trauma]] on the joint

* Omega-3 fatty acid

==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Rheumatology]]

Osteoarthritis overview

2020-05-13T16:22:12Z

Ayesha A. Khan:

[[File:TITLE.jpg|thumb|400x400px|'''Osteoarthritis of the Medial Side of the Knee.''']]
<div style="-webkit-user-select: none;">
{| class="infobox" style="position: fixed; top: 65%; right: 10px; margin: 0 0 0 0; border: 0; float: right;"
|-
| {{#ev:youtube|https://https://www.youtube.com/watch?v=sUOlmI-naFs|350}}
|-
|}
__NOTOC__
{{Osteoarthritis}}
{{CMG}}; {{AE}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com] [[User:DrMars|Mohammadmain Rezazadehsaatlou]]'''''<nowiki/>''''', [[User:Irfan Dotani|Irfan Dotani]]

=='''Overview'''==
'''Osteoarthritis / Osteoarthrosis''' (OA, also known as [[degenerative joint disease]], [[degenerative arthritis]], arthrosis or in more colloquial terms "wear and tear") is the most common form of arthritis, caused by wearing of the [[cartilage]] that covers and cushions joint spaces. As the cartilage wears away, the patient may experience pain described as "weight-bearing" whenever walking and standing. Due to the movement limitations caused by pain, regional muscles may experience [[atrophy]]. [[Ligament|Ligaments]] may become laxer as well due to this. OA is derived from the Greek word "''osteo''", meaning "of the bone", "''arthro''", meaning "joint", and "''itis''", meaning [[inflammation]], although inflammation is not a common finding in this regard. [[Osteoarthritis|OA]] possesses a great degree of variability in disease onset, progression, and severity. [[Osteoarthritis|OA]] is characterized by a variety of structural and functional impairments occurring in an involved joint. Destruction, degeneration, articular cartilage loss, and even the soft tissue involvement are the main pathological process of this disease. It can be diagnosed through radiographic evaluations. Moreover, clinical sign and symptoms are helpful in the final diagnosis of this disease. OA can be defined radiologically, clinically, or pathologically, with radiographic OA being considered as the reference standard. The symptoms that are consistently associated with OA are joint pain, stiffness, swelling, and limitation of joint function. Few individuals who present these symptoms may not demonstrate radiographic OA. However, others confirmed to have OA using radiographic techniques may not present with clinical manifestations of the disease. These unique characteristics have made it difficult to identify the underlying mechanisms contributing to the disease as well as the treatments for reducing the incidence and severity of the disease. In addition, the stimuli that may initiate the processes associated with OA are multifactorial and include occupational and non-occupational (e.g., genetics, obesity, age, etc.) factors. [[Osteoarthritis|OA]] affects nearly 43 million patients in [[United States]] and almost 15% of the world population, accounting for 25% of visits to [[Primary care physician|primary care physicians]], and half of all [[NSAID]] (Non-Steroidal Anti-Inflammatory Drugs) [[Medical prescription|prescriptions]]. It is estimated that 80% of the population will have [[Radiograph|radiographic]] evidence of OA by age 65, although only 60% of those will be There is no recent discovery of a cure for OA, as cartilage has not been induced to regenerate. However, if OA is caused by cartilage damage (for example as a result of an injury) Autologous Chondrocyte Implantation may be a possible treatment. Other treatments are with NSAIDs, local injections of [[glucocorticoid]] or [[hyaluronan]], and in severe cases, with [[joint replacement]] surgery. Many physicians have also reported good pain relief by treating ligaments (which is responsible for a bone to bone connection) with [[Prolotherapy]]. Clinical trials employing [[Tissue engineering|tissue-engineering]] methods have demonstrated regeneration of cartilage in damaged knees, including those that have progressed to osteoarthritis. Furthermore, in January 2007, Johns Hopkins University was offering to license a technology of this kind, listing several clinical competitors in its market analysis. Osteoarthritis is capable of influencing any joint in the human body; meanwhile, the most commonly affected joints are the knee and hip joints given that the degree of weight bearing required of these joints is immense. Other joints, such as the [[distal interphalangeal joints]] of the fingers and shoulder joints are also commonly affected as well. The economic burden of OA for United States economy is more than $60 billion per year; which has more economic pressure than [[rheumatoid arthritis]]. This cost can be considered into two subgroups: the medical related costs and the lost expediency of patients at work <ref name="pmid25586654">{{cite journal |vauthors=Peter WF, Dekker J, Tilbury C, Tordoir RL, Verdegaal SH, Onstenk R, Bénard MR, Vehmeijer SB, Fiocco M, Vermeulen HM, van der Linden-van der Zwaag HM, Nelissen RG, Vliet Vlieland TP |title=The association between comorbidities and pain, physical function and quality of life following hip and knee arthroplasty |journal=Rheumatol. Int. |volume=35 |issue=7 |pages=1233–41 |date=July 2015 |pmid=25586654 |pmc=4436688 |doi=10.1007/s00296-015-3211-7 |url=}}</ref>

=='''Historical Perspective'''==

The earliest descriptions of [[Osteoarthritis|OA]] were provided by Heberden and Haygarth in the 19th century. In the 1930s and 1940s, Dr. Stecher showed that there were two forms of OA: idiopathic and post-traumatic. [[Osteoarthritis overview#cite note-5|[5]]] In the 1950s, the connection between Heberden’s nodes and large joint OA were revealed by Kellgren and Moore. In this regard, the first x-ray grading system for OA was developed by Jonas Kellgren and John Lawrence in the 1950s. Surgical management of OA was developed in the 1960s by Drs. Charnley and McKee <ref name="pmid22632687">{{cite journal |vauthors=Suri P, Morgenroth DC, Hunter DJ |title=Epidemiology of osteoarthritis and associated comorbidities |journal=PM R |volume=4 |issue=5 Suppl |pages=S10–9 |date=May 2012 |pmid=22632687 |doi=10.1016/j.pmrj.2012.01.007 |url=}}</ref>

=='''Classification'''==

Osteoarthritis is radiographically classified depending on the degree of joint involvement. The Kellgren-Lawrence is a common method to classify the severity of OA in the knee using five different grades. This classification was proposed by Kellgren et al. in 1957 and was then accepted by WHO in 1961 <ref name="pmid25628884">{{cite journal |vauthors=Hardcastle SA, Dieppe P, Gregson CL, Davey Smith G, Tobias JH |title=Osteoarthritis and bone mineral density: are strong bones bad for joints? |journal=Bonekey Rep |volume=4 |issue= |pages=624 |date=2015 |pmid=25628884 |pmc=4303262 |doi=10.1038/bonekey.2014.119 |url=}}</ref>...

==Pathophysiology==
Osteoarthritis (OA) is a well-known [[degenerative joint disease]] influencing millions of people worldwide. Osteoarthritis is a complex disease caused by changes in the tissues' homeostasis of articular cartilages and subchondral bones. The cell/extracellular matrix (ECM) and their interactions play an important role in the pathophysiology of articular cartilage and the occurrence of Osteoarthritis. Consequently, the main feature of OA is that after this process is involved, the articular cartilages of the involved joint no longer will have a normal acting system because the destruction of the articular cartilages can no longer act as shock absorber. abnormal integrin expression alters cell/ECM signaling and modifies chondrocyte synthesis, with the following imbalance of destructive cytokines over regulatory factors. IL-1, TNF-alpha and other pro-catabolic cytokines activate the enzymatic degradation of cartilage matrix and are not counterbalanced by adequate synthesis of inhibitors. The main enzymes involved in ECM breakdown are metalloproteinases (MMPs), which are sequentially activated by an amplifying cascade. MMP activity is partially inhibited by the tissue inhibitors of MMPs (TIMPs), whose synthesis is low compared with MMP production in OA cartilage.

==Causes==
Osteoarthritis (OA) is a result of a variety of disorders that lead to structural and functional failure in involved joints. Osteoarthritis, traditionally, has been considered as a disease specifically related to articular cartilage. Presently, it's been proven that OA influencing the whole joint system consisted of capsule, and synovium, subchondral bone, cartilage, menisci, ligaments, and periarticular muscle.

==Differentiating {{PAGENAME}} from Other Diseases==
OA must be differentiated from other diseases that cause joint impairment and other related signs and symptoms such as [[Rheumatoid arthritis]], [[Gout]], Joint [[tuberculosis]].

==Epidemiology and Demographics==
OA is one of the most frequent diagnoses and is the leading cause of disability among the adult population in the USA. According to the National Health and Nutrition Examination Survey (NHANES), more than 26 million people in the USA were diagnosed with different forms of OA. The National Health Interview Survey (NHIS) reported that the 46.4 million Americans and 21.6% of Americans adults were diagnosed with arthritis. OA can involve any joint, but knees, hips, hands, are most common sites for this involvement.

==Risk Factors==
Osteoarthritis is a multifactorial disease and the interactions between systemic and local factors play important role in development and prognosis of OA.

==Screening==
Routine screening for osteoarthritis is not indicated unless the patient is symptomatic.

==Natural History, Complications, and Prognosis==
===Natural History===
The orthopedic surgeons are frequently asked by their patients regarding the final outcome and the prognosis of their diseased joint/joints caused by OA. Information about the natural history of OA is very important for upcoming determinations and planning for patients management. A small number of studies are available studied the role of the radiographic findings, joint congruence, or even the daily life activity on the OA progression.

===Complications===
OA is a leading cause of morbidity having significant effects on patients life and the health care system and even it could cause heavy economic burden. According to the American Academy of Orthopedic Surgeons report movement limitation are found in 80% of adults diagnosed with osteoarthritis. Meanwhile, 25% of these patients facing difficulties in their of daily living activities. 11% of them need personal care assistance and 14% required help with their routine needs.

===Prognosis===
Most osteoarthritis cases do stabilize. Some osteoarthritis cases progress. A small number of osteoarthritis patients improve spontaneously.

==Diagnosis==
===Diagnostic Criteria===
There is no specific signs and symptoms of osteoarthritis (OA) diagnosis. Although, osteoarthritis may be diagnosed based on several elements such as patients age, past medical history, and available symptoms. Osteoarthritis of the major joints is diagnosed using a combination of patients past medical history, physical examination, and variety of lab tests including imaging studies such as X-ray.

===History and Symptoms===
Patients' past medical history is the most useful tool for the osteoarthritis diagnosis.

===Physical Examination===
A physical examination following the medical history is necessary for medical doctors to reach an exact diagnosis. In OA, loss or limited range of motion in specific joints, swelling, tenderness, and bony growths in the surrounding area are the most important keys in physical examination of OA cases.

===Laboratory Findings===
If the OA diagnosis is in doubt, laboratory tests are used to help doctors get a confirmation regarding a suspected diagnosis of osteoarthritis.

===Imaging Findings===
X-Ray and CT-Scan are the most common tools used in this regard.

===Other Diagnostic Studies===

==Treatment==
===Medical Therapy===
Nonpharmacologic therapy is consisted of physical therapy and specific type of physical exercises, bracing and splinting. Physical therapy results in short-term pain reduction, and improvement in physical function in the diseased joint to preserve its the ability for daily tasks like walking, dressing, and even bathing. Having moderate activity strengthens the muscles around the diseased joint and this reduces stress and increases the stability of the joint system. On the other hand, resting is another important healing factor in OA. Bracing and splinting as other methods help to support painful or unstable joints. Using a cane can help decrease the weight pressure in diseased hip or knee, but it should be used on the contralateral side of the affected joint.

===Surgery===
Surgical interventions in OA cases should be considered when the symptoms have no response to the first line treatments because osteoarthritis symptoms can be successfully managed through [[Osteoarthritis medical therapy|non-surgical]] care. For some, however, if they are experiencing severe joint damage, extreme pain, or very restricted mobility, surgery may be a viable option in this regard. The main indication criteria for surgery in OA is pain and disabilities despite the medical treatments. The most common and effective surgical intervention are arthroscopic surgery, osteotomy, and arthroplasty (total joint replacement). Considering the potential benefits of surgery like pain relief, improved movement, increased patients status, and actual disease prognosis, it should be remembered that any surgical interventions have risks. Meanwhile, overweight patients or patients with co-morbidities have higher risk of operation. The current joint prostheses have an expected functional usage for almost 15 to 20 years.

===Prevention===

Primary prevention for OA include :
* [[Weight loss]]
* Physical activity
* Injury prevention
* Control infectious disease
* Avoidance of [[trauma]] on the joint

* Omega-3 fatty acid

==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Rheumatology]]

Osteoarthritis overview

2020-05-13T16:20:50Z

Ayesha A. Khan: /* Pathophysiology */

[[File:TITLE.jpg|thumb|400x400px|'''Osteoarthritis of the Medial Side of the Knee.''']]
<div style="-webkit-user-select: none;">
{| class="infobox" style="position: fixed; top: 65%; right: 10px; margin: 0 0 0 0; border: 0; float: right;"
|-
| {{#ev:youtube|https://https://www.youtube.com/watch?v=sUOlmI-naFs|350}}
|-
|}
__NOTOC__
{{Osteoarthritis}}
{{CMG}}; {{AE}}[[User:DrMars|Mohammadmain Rezazadehsaatlou]]'''''<nowiki/>''''', [[User:Irfan Dotani|Irfan Dotani]]

=='''Overview'''==
'''Osteoarthritis / Osteoarthrosis''' (OA, also known as [[degenerative joint disease]], [[degenerative arthritis]], arthrosis or in more colloquial terms "wear and tear") is the most common form of arthritis, caused by wearing of the [[cartilage]] that covers and cushions joint spaces. As the cartilage wears away, the patient may experience pain described as "weight-bearing" whenever walking and standing. Due to the movement limitations caused by pain, regional muscles may experience [[atrophy]]. [[Ligament|Ligaments]] may become laxer as well due to this. OA is derived from the Greek word "''osteo''", meaning "of the bone", "''arthro''", meaning "joint", and "''itis''", meaning [[inflammation]], although inflammation is not a common finding in this regard. [[Osteoarthritis|OA]] possesses a great degree of variability in disease onset, progression, and severity. [[Osteoarthritis|OA]] is characterized by a variety of structural and functional impairments occurring in an involved joint. Destruction, degeneration, articular cartilage loss, and even the soft tissue involvement are the main pathological process of this disease. It can be diagnosed through radiographic evaluations. Moreover, clinical sign and symptoms are helpful in the final diagnosis of this disease. OA can be defined radiologically, clinically, or pathologically, with radiographic OA being considered as the reference standard. The symptoms that are consistently associated with OA are joint pain, stiffness, swelling, and limitation of joint function. Few individuals who present these symptoms may not demonstrate radiographic OA. However, others confirmed to have OA using radiographic techniques may not present with clinical manifestations of the disease. These unique characteristics have made it difficult to identify the underlying mechanisms contributing to the disease as well as the treatments for reducing the incidence and severity of the disease. In addition, the stimuli that may initiate the processes associated with OA are multifactorial and include occupational and non-occupational (e.g., genetics, obesity, age, etc.) factors. [[Osteoarthritis|OA]] affects nearly 43 million patients in [[United States]] and almost 15% of the world population, accounting for 25% of visits to [[Primary care physician|primary care physicians]], and half of all [[NSAID]] (Non-Steroidal Anti-Inflammatory Drugs) [[Medical prescription|prescriptions]]. It is estimated that 80% of the population will have [[Radiograph|radiographic]] evidence of OA by age 65, although only 60% of those will be There is no recent discovery of a cure for OA, as cartilage has not been induced to regenerate. However, if OA is caused by cartilage damage (for example as a result of an injury) Autologous Chondrocyte Implantation may be a possible treatment. Other treatments are with NSAIDs, local injections of [[glucocorticoid]] or [[hyaluronan]], and in severe cases, with [[joint replacement]] surgery. Many physicians have also reported good pain relief by treating ligaments (which is responsible for a bone to bone connection) with [[Prolotherapy]]. Clinical trials employing [[Tissue engineering|tissue-engineering]] methods have demonstrated regeneration of cartilage in damaged knees, including those that have progressed to osteoarthritis. Furthermore, in January 2007, Johns Hopkins University was offering to license a technology of this kind, listing several clinical competitors in its market analysis. Osteoarthritis is capable of influencing any joint in the human body; meanwhile, the most commonly affected joints are the knee and hip joints given that the degree of weight bearing required of these joints is immense. Other joints, such as the [[distal interphalangeal joints]] of the fingers and shoulder joints are also commonly affected as well. The economic burden of OA for United States economy is more than $60 billion per year; which has more economic pressure than [[rheumatoid arthritis]]. This cost can be considered into two subgroups: the medical related costs and the lost expediency of patients at work <ref name="pmid25586654">{{cite journal |vauthors=Peter WF, Dekker J, Tilbury C, Tordoir RL, Verdegaal SH, Onstenk R, Bénard MR, Vehmeijer SB, Fiocco M, Vermeulen HM, van der Linden-van der Zwaag HM, Nelissen RG, Vliet Vlieland TP |title=The association between comorbidities and pain, physical function and quality of life following hip and knee arthroplasty |journal=Rheumatol. Int. |volume=35 |issue=7 |pages=1233–41 |date=July 2015 |pmid=25586654 |pmc=4436688 |doi=10.1007/s00296-015-3211-7 |url=}}</ref>

=='''Historical Perspective'''==

The earliest descriptions of [[Osteoarthritis|OA]] were provided by Heberden and Haygarth in the 19th century. In the 1930s and 1940s, Dr. Stecher showed that there were two forms of OA: idiopathic and post-traumatic. [[Osteoarthritis overview#cite note-5|[5]]] In the 1950s, the connection between Heberden’s nodes and large joint OA were revealed by Kellgren and Moore. In this regard, the first x-ray grading system for OA was developed by Jonas Kellgren and John Lawrence in the 1950s. Surgical management of OA was developed in the 1960s by Drs. Charnley and McKee <ref name="pmid22632687">{{cite journal |vauthors=Suri P, Morgenroth DC, Hunter DJ |title=Epidemiology of osteoarthritis and associated comorbidities |journal=PM R |volume=4 |issue=5 Suppl |pages=S10–9 |date=May 2012 |pmid=22632687 |doi=10.1016/j.pmrj.2012.01.007 |url=}}</ref>

=='''Classification'''==

Osteoarthritis is radiographically classified depending on the degree of joint involvement. The Kellgren-Lawrence is a common method to classify the severity of OA in the knee using five different grades. This classification was proposed by Kellgren et al. in 1957 and was then accepted by WHO in 1961 <ref name="pmid25628884">{{cite journal |vauthors=Hardcastle SA, Dieppe P, Gregson CL, Davey Smith G, Tobias JH |title=Osteoarthritis and bone mineral density: are strong bones bad for joints? |journal=Bonekey Rep |volume=4 |issue= |pages=624 |date=2015 |pmid=25628884 |pmc=4303262 |doi=10.1038/bonekey.2014.119 |url=}}</ref>...

==Pathophysiology==
Osteoarthritis (OA) is a well-known [[degenerative joint disease]] influencing millions of people worldwide. Osteoarthritis is a complex disease caused by changes in the tissues' homeostasis of articular cartilages and subchondral bones. The cell/extracellular matrix (ECM) and their interactions play an important role in the pathophysiology of articular cartilage and the occurrence of Osteoarthritis. Consequently, the main feature of OA is that after this process is involved, the articular cartilages of the involved joint no longer will have a normal acting system because the destruction of the articular cartilages can no longer act as shock absorber. abnormal integrin expression alters cell/ECM signaling and modifies chondrocyte synthesis, with the following imbalance of destructive cytokines over regulatory factors. IL-1, TNF-alpha and other pro-catabolic cytokines activate the enzymatic degradation of cartilage matrix and are not counterbalanced by adequate synthesis of inhibitors. The main enzymes involved in ECM breakdown are metalloproteinases (MMPs), which are sequentially activated by an amplifying cascade. MMP activity is partially inhibited by the tissue inhibitors of MMPs (TIMPs), whose synthesis is low compared with MMP production in OA cartilage.

==Causes==
Osteoarthritis (OA) is a result of a variety of disorders that lead to structural and functional failure in involved joints. Osteoarthritis, traditionally, has been considered as a disease specifically related to articular cartilage. Presently, it's been proven that OA influencing the whole joint system consisted of capsule, and synovium, subchondral bone, cartilage, menisci, ligaments, and periarticular muscle.

==Differentiating {{PAGENAME}} from Other Diseases==
OA must be differentiated from other diseases that cause joint impairment and other related signs and symptoms such as [[Rheumatoid arthritis]], [[Gout]], Joint [[tuberculosis]].

==Epidemiology and Demographics==
OA is one of the most frequent diagnoses and is the leading cause of disability among the adult population in the USA. According to the National Health and Nutrition Examination Survey (NHANES), more than 26 million people in the USA were diagnosed with different forms of OA. The National Health Interview Survey (NHIS) reported that the 46.4 million Americans and 21.6% of Americans adults were diagnosed with arthritis. OA can involve any joint, but knees, hips, hands, are most common sites for this involvement.

==Risk Factors==
Osteoarthritis is a multifactorial disease and the interactions between systemic and local factors play important role in development and prognosis of OA.

==Screening==
Routine screening for osteoarthritis is not indicated unless the patient is symptomatic.

==Natural History, Complications, and Prognosis==
===Natural History===
The orthopedic surgeons are frequently asked by their patients regarding the final outcome and the prognosis of their diseased joint/joints caused by OA. Information about the natural history of OA is very important for upcoming determinations and planning for patients management. A small number of studies are available studied the role of the radiographic findings, joint congruence, or even the daily life activity on the OA progression.

===Complications===
OA is a leading cause of morbidity having significant effects on patients life and the health care system and even it could cause heavy economic burden. According to the American Academy of Orthopedic Surgeons report movement limitation are found in 80% of adults diagnosed with osteoarthritis. Meanwhile, 25% of these patients facing difficulties in their of daily living activities. 11% of them need personal care assistance and 14% required help with their routine needs.

===Prognosis===
Most osteoarthritis cases do stabilize. Some osteoarthritis cases progress. A small number of osteoarthritis patients improve spontaneously.

==Diagnosis==
===Diagnostic Criteria===
There is no specific signs and symptoms of osteoarthritis (OA) diagnosis. Although, osteoarthritis may be diagnosed based on several elements such as patients age, past medical history, and available symptoms. Osteoarthritis of the major joints is diagnosed using a combination of patients past medical history, physical examination, and variety of lab tests including imaging studies such as X-ray.

===History and Symptoms===
Patients' past medical history is the most useful tool for the osteoarthritis diagnosis.

===Physical Examination===
A physical examination following the medical history is necessary for medical doctors to reach an exact diagnosis. In OA, loss or limited range of motion in specific joints, swelling, tenderness, and bony growths in the surrounding area are the most important keys in physical examination of OA cases.

===Laboratory Findings===
If the OA diagnosis is in doubt, laboratory tests are used to help doctors get a confirmation regarding a suspected diagnosis of osteoarthritis.

===Imaging Findings===
X-Ray and CT-Scan are the most common tools used in this regard.

===Other Diagnostic Studies===

==Treatment==
===Medical Therapy===
Nonpharmacologic therapy is consisted of physical therapy and specific type of physical exercises, bracing and splinting. Physical therapy results in short-term pain reduction, and improvement in physical function in the diseased joint to preserve its the ability for daily tasks like walking, dressing, and even bathing. Having moderate activity strengthens the muscles around the diseased joint and this reduces stress and increases the stability of the joint system. On the other hand, resting is another important healing factor in OA. Bracing and splinting as other methods help to support painful or unstable joints. Using a cane can help decrease the weight pressure in diseased hip or knee, but it should be used on the contralateral side of the affected joint.

===Surgery===
Surgical interventions in OA cases should be considered when the symptoms have no response to the first line treatments because osteoarthritis symptoms can be successfully managed through [[Osteoarthritis medical therapy|non-surgical]] care. For some, however, if they are experiencing severe joint damage, extreme pain, or very restricted mobility, surgery may be a viable option in this regard. The main indication criteria for surgery in OA is pain and disabilities despite the medical treatments. The most common and effective surgical intervention are arthroscopic surgery, osteotomy, and arthroplasty (total joint replacement). Considering the potential benefits of surgery like pain relief, improved movement, increased patients status, and actual disease prognosis, it should be remembered that any surgical interventions have risks. Meanwhile, overweight patients or patients with co-morbidities have higher risk of operation. The current joint prostheses have an expected functional usage for almost 15 to 20 years.

===Prevention===

Primary prevention for OA include :
* [[Weight loss]]
* Physical activity
* Injury prevention
* Control infectious disease
* Avoidance of [[trauma]] on the joint

* Omega-3 fatty acid

==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Rheumatology]]

User:Ayesha A. Khan

2020-05-12T18:28:13Z

Ayesha A. Khan: /* Contact */

[[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com]

Dr. Ayesha Khan, MD is an Internist in Cleveland, OH.

==Contact==
Email: Ayesha.khan@stvincentcharity.com

ORCID: 0000-0001-6479-5939

==Specialties==
Internist, Rheumatology, Nephrology.

==Hospital affiliations==

*Kindred Hospital-Cleveland-Gateway
*Outreach Professional Services Inc
*St. Vincent Charity Medical Center

User:Ayesha A. Khan

2020-05-12T18:27:29Z

Ayesha A. Khan:

[[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com]

Dr. Ayesha Khan, MD is an Internist in Cleveland, OH.

==Contact==
Email: Ayesha.khan@stvincentcharity.com
ORCID: 0000-0001-6479-5939
==Specialties==
Internist, Rheumatology, Nephrology.

==Hospital affiliations==

*Kindred Hospital-Cleveland-Gateway
*Outreach Professional Services Inc
*St. Vincent Charity Medical Center

Polymyalgia rheumatica overview

2020-05-12T18:14:41Z

Ayesha A. Khan:

__NOTOC__
{{Polymyalgia rheumatica}}
{{CMG}}; {{AE}} {{CZ}}; [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com] Ujjwal Rastogi, MBBS [mailto:urastogi@perfuse.org]; {{Rim}}; {{AEL}}

==Overview==
Polymyalgia rheumatica (PMR) is a chronic inflammatory disease that involves the [[articular]] and periarticular parts of the cervical region, [[shoulder girdle]] and [[pelvic girdle]]. PMR affects subjects over the age of 50 years and it is characterized by [[pain]] and stiffness in the [[neck]], [[Shoulder|shoulders]], upper arms, [[Hip (anatomy)|hip]], and [[Thigh|thighs]]. Although [[myalgia]] is one of the symptoms of PMR, there is no [[inflammation]] of the [[muscle]]s; instead, PMR is a disease of the [[joint]] that causes [[synovitis]]. The diagnosis of PMR relies on the clinical findings and laboratory evidence of systemic [[inflammation]]. PMR is associated with [[giant cell arteritis]]. The cause of PMR is unknown but it has been suggested that both [[Genetics|genetic]] and environmental factors are implicated. The mainstay of treatment of PMR is [[steroid]] therapy.

==Historical Perspective==
Polymyalgia rheumatica was first described in 1888 by Bruce William as "senile rheumatic gout". The disease was referred to as "polymyalgia rheumatica" by Stuart Barber in 1957 in his article entitled "myalgic syndrome with constitutional effects; polymyalgia rheumatica".

==Classification==
There is no established system for the classification of polymyalgia rheumatica.

==Pathophysiology==
Polymyalgia rheumatica (PMR) is a chronic [[Inflammation|inflammatory]] disease of the [[articular]] and periarticular structures of the [[cervical]] region, [[Pectoral girdle|shoulder girdle]] and [[Hip (anatomy)|hip girdle]]. The underlying pathophysiology of PMR remains unknown. It has been hypothesized that genetic and environmental factors are implicated, particularly due to the seasonal and geographical differences in the prevalence of this disease. It has also been hypothesized that PMR is associated with [[Infection|infections]] such as [[parainfluenza virus type 1]], [[mycoplasma pneumoniae]], [[chlamydia pneumoniae]], and [[parvovirus B19]]. In addition, histological examinations of [[synovial]] [[Biopsy|biopsies]] of affected individuals reveal mild [[synovitis]] with predominance of [[CD4 T cells]] and [[macrophages]]. Although [[myalgia]] is a symptom of PMR, there is no [[inflammation]] of the [[Muscle|muscles]].The [[senescence]] of the [[immune systems]] as demonstrated by the loss of the [[CD28]] on [[CD4+ T]] [[senescent cells]] may be responsible for aberrant [[immune]] responses in [[PMR]]. [[Adaptive]] [[immune]] alterations also occurs in [[PMR]] mainly represented by the activation of [[Th17 cells]], mainly driven by the increased [[IL-6]] levels. An altered distribution and phenotype of B cells also occurs in [[PMR]] even in the absence of a clear [[autoimmune]] response. Local activation of [[myeloid]] and [[endothelial cells]] has been also demonstrated in the non-inflamed arteries and inflamed synovial tissues of PMR patients.

==Causes==
There is no specific cause for polymyalgia rheumatica. However, there are possible theories about the causes which include [[Inflammation|inflammatory]] joint lining attack, [[Virus|viral infections]], and [[Biological inheritance|genetic inheritance]] of [[HLA-DR4]].

==Differentiating Polymyalgia Rheumatica from other Diseases==
PMR must be differentiated from other conditions such as late onset [[rheumatoid arthritis]], [[polymyositis]], [[dermatomyositis]], [[fibromyalgia]], and [[remitting seronegative symmetrical synovitis with pitting edema]].

==Epidemiology and Demographics==
Polymyalgia rheumatica (PMR) affects mostly subjects who are more than 50 years of age. The prevalence of PMR is highest among subjects from Scandinavian countries and those from northern European origin.

==Risk Factors==
Age, female sex, Scandinavia and northern Europe origin are risk factors for polymyalgia rheumatica. Other risk factors include [[smoking]], [[sun exposure]], [[Infection|infections]], and nulliparity.

== Screening ==
There is insufficient evidence to recommend routine screening for polymyalgia rheumatica.

==Natural History, Complications and Prognosis==
Polymyalgia rheumatica (PMR) affects the quality of life of the patients. The [[pain]] and stiffness in the proximal [[joints]] might lead to sleep disturbance as well as an inability to do regular daily activities such as getting dressed and getting out of a chair. The symptoms begin rapidly and last for weeks. Once the [[steroid]] treatment is initiated, the symptoms resolve rapidly within few days. In fact, the rapid resolution of symptoms with the [[steroid]] therapy reinforces the diagnosis of PMR. The [[steroid]] treatment can be associated with complications such as [[weight gain]] and [[bone fracture]]. Approximately 40 to 50% of subjects experience a relapse of the symptoms. PMR is associated with [[giant cell arteritis]].

==Diagnosis==
===Diagnostic Study of Choice===
The diagnosis of polymyalgia rheumatica (PMR) is mostly clinical and it is supported with specific findings on laboratory tests and [[ultrasound]] of the affected [[joints]]. The European League Against Rheumatism/American College of Rheumatology collaborative initiative developed a provisional classification criteria for PMR. The following criteria are required for the diagnosis of PMR: age more than 50 years, bilateral [[shoulder pain]], and elevated [[C-reactive protein]] (CRP) and/or [[erythrocyte sedimentation rate]] (ESR).

===History and Symptoms===
Polymyalgia rheumatica (PMR) is typically characterized by symmetrical [[pain]] and morning stiffness in the proximal [[Joint|joints]] and [[Limb (anatomy)|limbs]], including the [[neck]], the [[shoulder girdle]], the [[pelvic girdle]], the [[lower back]], and the [[Thigh|thighs]]. In some patients, there is involvement of the distal parts of the body such as peripheral [[synovitis]] or [[arthritis]]. Constitutional symptoms can also be present, and they include [[fever]], [[fatigue]], [[loss of appetite]], and [[weight loss]]. There is an association between PMR and [[giant cell arteritis]] which can present with one or more of the following symptoms that include [[headaches]], scalp tenderness, [[jaw claudication]], [[fever]], or distorted vision.

===Physical Examination===
Physical examination of patients with polymyalgia rheumatica reveals limitation of the active and passive [[range of motion]] of the affected [[joint]]. There is no true [[muscle weakness]]. There are no changes in the [[Joint|joints]]. [[Ophthalmoscope|Ophthalmoscopic]] exams in patients with polymyalgia rheumatica associated with [[Temporal arteritis|giant cell arteritis]] might be abnormal.

===Laboratory Findings===
Polymyalgia rheumatica (PMR) is a clinical diagnosis that is supported by laboratory tests. Elevation of [[C-reactive protein]] (CRP) and/or [[erythrocyte sedimentation rate]] (ESR) is essential for the diagnosis of PMR.

=== Electrocardiogram ===
There are no EKG findings associated with polymyalgia rheumatica.

===X Ray===
There are no x ray findings associated with polymyalgia rheumatica.

=== Echocardiography and Ultrasound ===
There are no echocardiography findings associated with polymyalgia rheumatica. [[Ultrasound]] exam is important for the diagnosis of polymyalgia rheumatica (PMR). It can reveal evidence of [[bursitis]], [[synovitis]] or [[tenosynovitis]] in the affected areas.

===CT Scan===
There are no CT findings associated with polymyalgia rheumatica.

===MRI===
[[MRI]] is used for the assessment of [[bursitis]], [[synovitis]], and [[tenosynovitis]] among patients with polymyalgia rheumatica (PMR). [[MRI]] is more sensitive than [[ultrasonography]] for the evaluation of iliopsoas [[bursitis]] and hip [[synovitis]]. A study has demonstrated that [[MRI]] of the shoulders facilitates the proper diagnosis in patients with the typical proximal symptoms of [[PMR]] with normal [[ESR]] values.

===Other Imaging Findings===
There are no other imaging findings associated with polymyalgia rheumatica.

===Other Diagnostic Studies===
A [[muscle biopsy]] might be performed to differentiate polymyalgia rheumatica (PMR) from other diseases. In addition, [[temporal artery]] biopsy is required when a subject with PMR have symptoms suggestive of [[giant cell arteritis]].

==Treatment==

===Medical Therapy===
The mainstay of treatment of polymyalgia rheumatica (PMR) is low dose [[glucocorticoids]], typically [[prednisone]] or [[prednisolone]]. The starting dose of the [[glucocorticoid]] treatment is 12.5-15 mg daily for 2 to 4 weeks after which the treatment should be slowly tapered. The average duration of the treatment with [[glucocorticoids]] is 1 to 2 years; nevertheless, longer [[corticosteroids]] regimens might be necessary among patients who experience relapse of the symptoms. Prophylaxis for [[osteoporosis]] with [[calcium]] and [[vitamin D]] should be started with the [[steroid]] therapy.

===Surgery===
Surgical intervention is not recommended for the management of polymyalgia rheumatica.

===Primary Prevention===
There are no established measures for the primary prevention of polymyalgia rheumatica.

===Secondary Prevention===
There are no established measures for the secondary prevention of polymyalgia rheumatica.

==External links==
* [http://www.arthritis.ca/types%20of%20arthritis/polymyalgia%20rheumatica/default.asp?s=1 Polymyalgia Rheumatica] - The Arthritis Society
* [http://www.rheumatology.org/public/factsheets/pmr_new2.asp Patient Education - Polymyalgia Rheumatica] - American College of Rheumatology

[[Category:Medicine]]
[[Category:Rheumatology]]
[[Category:Up-To-Date]]
[[Category:Primary care]]

Polymyalgia rheumatica overview

2020-05-12T18:12:33Z

Ayesha A. Khan: /* Pathophysiology */

__NOTOC__
{{Polymyalgia rheumatica}}
{{CMG}}; {{AE}} {{CZ}}; Ujjwal Rastogi, MBBS [mailto:urastogi@perfuse.org]; {{Rim}}; {{AEL}}

==Overview==
Polymyalgia rheumatica (PMR) is a chronic inflammatory disease that involves the [[articular]] and periarticular parts of the cervical region, [[shoulder girdle]] and [[pelvic girdle]]. PMR affects subjects over the age of 50 years and it is characterized by [[pain]] and stiffness in the [[neck]], [[Shoulder|shoulders]], upper arms, [[Hip (anatomy)|hip]], and [[Thigh|thighs]]. Although [[myalgia]] is one of the symptoms of PMR, there is no [[inflammation]] of the [[muscle]]s; instead, PMR is a disease of the [[joint]] that causes [[synovitis]]. The diagnosis of PMR relies on the clinical findings and laboratory evidence of systemic [[inflammation]]. PMR is associated with [[giant cell arteritis]]. The cause of PMR is unknown but it has been suggested that both [[Genetics|genetic]] and environmental factors are implicated. The mainstay of treatment of PMR is [[steroid]] therapy.

==Historical Perspective==
Polymyalgia rheumatica was first described in 1888 by Bruce William as "senile rheumatic gout". The disease was referred to as "polymyalgia rheumatica" by Stuart Barber in 1957 in his article entitled "myalgic syndrome with constitutional effects; polymyalgia rheumatica".

==Classification==
There is no established system for the classification of polymyalgia rheumatica.

==Pathophysiology==
Polymyalgia rheumatica (PMR) is a chronic [[Inflammation|inflammatory]] disease of the [[articular]] and periarticular structures of the [[cervical]] region, [[Pectoral girdle|shoulder girdle]] and [[Hip (anatomy)|hip girdle]]. The underlying pathophysiology of PMR remains unknown. It has been hypothesized that genetic and environmental factors are implicated, particularly due to the seasonal and geographical differences in the prevalence of this disease. It has also been hypothesized that PMR is associated with [[Infection|infections]] such as [[parainfluenza virus type 1]], [[mycoplasma pneumoniae]], [[chlamydia pneumoniae]], and [[parvovirus B19]]. In addition, histological examinations of [[synovial]] [[Biopsy|biopsies]] of affected individuals reveal mild [[synovitis]] with predominance of [[CD4 T cells]] and [[macrophages]]. Although [[myalgia]] is a symptom of PMR, there is no [[inflammation]] of the [[Muscle|muscles]].The [[senescence]] of the [[immune systems]] as demonstrated by the loss of the [[CD28]] on [[CD4+ T]] [[senescent cells]] may be responsible for aberrant [[immune]] responses in [[PMR]]. [[Adaptive]] [[immune]] alterations also occurs in [[PMR]] mainly represented by the activation of [[Th17 cells]], mainly driven by the increased [[IL-6]] levels. An altered distribution and phenotype of B cells also occurs in [[PMR]] even in the absence of a clear [[autoimmune]] response. Local activation of [[myeloid]] and [[endothelial cells]] has been also demonstrated in the non-inflamed arteries and inflamed synovial tissues of PMR patients.

==Causes==
There is no specific cause for polymyalgia rheumatica. However, there are possible theories about the causes which include [[Inflammation|inflammatory]] joint lining attack, [[Virus|viral infections]], and [[Biological inheritance|genetic inheritance]] of [[HLA-DR4]].

==Differentiating Polymyalgia Rheumatica from other Diseases==
PMR must be differentiated from other conditions such as late onset [[rheumatoid arthritis]], [[polymyositis]], [[dermatomyositis]], [[fibromyalgia]], and [[remitting seronegative symmetrical synovitis with pitting edema]].

==Epidemiology and Demographics==
Polymyalgia rheumatica (PMR) affects mostly subjects who are more than 50 years of age. The prevalence of PMR is highest among subjects from Scandinavian countries and those from northern European origin.

==Risk Factors==
Age, female sex, Scandinavia and northern Europe origin are risk factors for polymyalgia rheumatica. Other risk factors include [[smoking]], [[sun exposure]], [[Infection|infections]], and nulliparity.

== Screening ==
There is insufficient evidence to recommend routine screening for polymyalgia rheumatica.

==Natural History, Complications and Prognosis==
Polymyalgia rheumatica (PMR) affects the quality of life of the patients. The [[pain]] and stiffness in the proximal [[joints]] might lead to sleep disturbance as well as an inability to do regular daily activities such as getting dressed and getting out of a chair. The symptoms begin rapidly and last for weeks. Once the [[steroid]] treatment is initiated, the symptoms resolve rapidly within few days. In fact, the rapid resolution of symptoms with the [[steroid]] therapy reinforces the diagnosis of PMR. The [[steroid]] treatment can be associated with complications such as [[weight gain]] and [[bone fracture]]. Approximately 40 to 50% of subjects experience a relapse of the symptoms. PMR is associated with [[giant cell arteritis]].

==Diagnosis==
===Diagnostic Study of Choice===
The diagnosis of polymyalgia rheumatica (PMR) is mostly clinical and it is supported with specific findings on laboratory tests and [[ultrasound]] of the affected [[joints]]. The European League Against Rheumatism/American College of Rheumatology collaborative initiative developed a provisional classification criteria for PMR. The following criteria are required for the diagnosis of PMR: age more than 50 years, bilateral [[shoulder pain]], and elevated [[C-reactive protein]] (CRP) and/or [[erythrocyte sedimentation rate]] (ESR).

===History and Symptoms===
Polymyalgia rheumatica (PMR) is typically characterized by symmetrical [[pain]] and morning stiffness in the proximal [[Joint|joints]] and [[Limb (anatomy)|limbs]], including the [[neck]], the [[shoulder girdle]], the [[pelvic girdle]], the [[lower back]], and the [[Thigh|thighs]]. In some patients, there is involvement of the distal parts of the body such as peripheral [[synovitis]] or [[arthritis]]. Constitutional symptoms can also be present, and they include [[fever]], [[fatigue]], [[loss of appetite]], and [[weight loss]]. There is an association between PMR and [[giant cell arteritis]] which can present with one or more of the following symptoms that include [[headaches]], scalp tenderness, [[jaw claudication]], [[fever]], or distorted vision.

===Physical Examination===
Physical examination of patients with polymyalgia rheumatica reveals limitation of the active and passive [[range of motion]] of the affected [[joint]]. There is no true [[muscle weakness]]. There are no changes in the [[Joint|joints]]. [[Ophthalmoscope|Ophthalmoscopic]] exams in patients with polymyalgia rheumatica associated with [[Temporal arteritis|giant cell arteritis]] might be abnormal.

===Laboratory Findings===
Polymyalgia rheumatica (PMR) is a clinical diagnosis that is supported by laboratory tests. Elevation of [[C-reactive protein]] (CRP) and/or [[erythrocyte sedimentation rate]] (ESR) is essential for the diagnosis of PMR.

=== Electrocardiogram ===
There are no EKG findings associated with polymyalgia rheumatica.

===X Ray===
There are no x ray findings associated with polymyalgia rheumatica.

=== Echocardiography and Ultrasound ===
There are no echocardiography findings associated with polymyalgia rheumatica. [[Ultrasound]] exam is important for the diagnosis of polymyalgia rheumatica (PMR). It can reveal evidence of [[bursitis]], [[synovitis]] or [[tenosynovitis]] in the affected areas.

===CT Scan===
There are no CT findings associated with polymyalgia rheumatica.

===MRI===
[[MRI]] is used for the assessment of [[bursitis]], [[synovitis]], and [[tenosynovitis]] among patients with polymyalgia rheumatica (PMR). [[MRI]] is more sensitive than [[ultrasonography]] for the evaluation of iliopsoas [[bursitis]] and hip [[synovitis]]. A study has demonstrated that [[MRI]] of the shoulders facilitates the proper diagnosis in patients with the typical proximal symptoms of [[PMR]] with normal [[ESR]] values.

===Other Imaging Findings===
There are no other imaging findings associated with polymyalgia rheumatica.

===Other Diagnostic Studies===
A [[muscle biopsy]] might be performed to differentiate polymyalgia rheumatica (PMR) from other diseases. In addition, [[temporal artery]] biopsy is required when a subject with PMR have symptoms suggestive of [[giant cell arteritis]].

==Treatment==

===Medical Therapy===
The mainstay of treatment of polymyalgia rheumatica (PMR) is low dose [[glucocorticoids]], typically [[prednisone]] or [[prednisolone]]. The starting dose of the [[glucocorticoid]] treatment is 12.5-15 mg daily for 2 to 4 weeks after which the treatment should be slowly tapered. The average duration of the treatment with [[glucocorticoids]] is 1 to 2 years; nevertheless, longer [[corticosteroids]] regimens might be necessary among patients who experience relapse of the symptoms. Prophylaxis for [[osteoporosis]] with [[calcium]] and [[vitamin D]] should be started with the [[steroid]] therapy.

===Surgery===
Surgical intervention is not recommended for the management of polymyalgia rheumatica.

===Primary Prevention===
There are no established measures for the primary prevention of polymyalgia rheumatica.

===Secondary Prevention===
There are no established measures for the secondary prevention of polymyalgia rheumatica.

==External links==
* [http://www.arthritis.ca/types%20of%20arthritis/polymyalgia%20rheumatica/default.asp?s=1 Polymyalgia Rheumatica] - The Arthritis Society
* [http://www.rheumatology.org/public/factsheets/pmr_new2.asp Patient Education - Polymyalgia Rheumatica] - American College of Rheumatology

[[Category:Medicine]]
[[Category:Rheumatology]]
[[Category:Up-To-Date]]
[[Category:Primary care]]

Polymyalgia rheumatica overview

2020-05-12T18:09:40Z

Ayesha A. Khan: /* Pathophysiology */

__NOTOC__
{{Polymyalgia rheumatica}}
{{CMG}}; {{AE}} {{CZ}}; Ujjwal Rastogi, MBBS [mailto:urastogi@perfuse.org]; {{Rim}}; {{AEL}}

==Overview==
Polymyalgia rheumatica (PMR) is a chronic inflammatory disease that involves the [[articular]] and periarticular parts of the cervical region, [[shoulder girdle]] and [[pelvic girdle]]. PMR affects subjects over the age of 50 years and it is characterized by [[pain]] and stiffness in the [[neck]], [[Shoulder|shoulders]], upper arms, [[Hip (anatomy)|hip]], and [[Thigh|thighs]]. Although [[myalgia]] is one of the symptoms of PMR, there is no [[inflammation]] of the [[muscle]]s; instead, PMR is a disease of the [[joint]] that causes [[synovitis]]. The diagnosis of PMR relies on the clinical findings and laboratory evidence of systemic [[inflammation]]. PMR is associated with [[giant cell arteritis]]. The cause of PMR is unknown but it has been suggested that both [[Genetics|genetic]] and environmental factors are implicated. The mainstay of treatment of PMR is [[steroid]] therapy.

==Historical Perspective==
Polymyalgia rheumatica was first described in 1888 by Bruce William as "senile rheumatic gout". The disease was referred to as "polymyalgia rheumatica" by Stuart Barber in 1957 in his article entitled "myalgic syndrome with constitutional effects; polymyalgia rheumatica".

==Classification==
There is no established system for the classification of polymyalgia rheumatica.

==Pathophysiology==
Polymyalgia rheumatica (PMR) is a chronic [[Inflammation|inflammatory]] disease of the [[articular]] and periarticular structures of the [[cervical]] region, [[Pectoral girdle|shoulder girdle]] and [[Hip (anatomy)|hip girdle]]. The underlying pathophysiology of PMR remains unknown. It has been hypothesized that genetic and environmental factors are implicated, particularly due to the seasonal and geographical differences in the prevalence of this disease. It has also been hypothesized that PMR is associated with [[Infection|infections]] such as [[parainfluenza virus type 1]], [[mycoplasma pneumoniae]], [[chlamydia pneumoniae]], and [[parvovirus B19]]. In addition, histological examinations of [[synovial]] [[Biopsy|biopsies]] of affected individuals reveal mild [[synovitis]] with predominance of [[CD4 T cells]] and [[macrophages]]. Although [[myalgia]] is a symptom of PMR, there is no [[inflammation]] of the [[Muscle|muscles]].The [[senescence]] of the immune systems as demonstrated by the loss of the CD28 on CD4+ T senescent cells may be responsible for aberrant immune responses in PMR. Adaptive immune alterations also occurs in PMR mainly represented by the activation of Th17 cells, mainly driven by the increased IL-6 levels. An altered distribution and phenotype of B cells also occurs in PMR even in the absence of a clear autoimmune response. Local activation of myeloid and endothelial cells has been also demonstrated in the non-inflamed arteries and inflamed synovial tissues of PMR patients.

==Causes==
There is no specific cause for polymyalgia rheumatica. However, there are possible theories about the causes which include [[Inflammation|inflammatory]] joint lining attack, [[Virus|viral infections]], and [[Biological inheritance|genetic inheritance]] of [[HLA-DR4]].

==Differentiating Polymyalgia Rheumatica from other Diseases==
PMR must be differentiated from other conditions such as late onset [[rheumatoid arthritis]], [[polymyositis]], [[dermatomyositis]], [[fibromyalgia]], and [[remitting seronegative symmetrical synovitis with pitting edema]].

==Epidemiology and Demographics==
Polymyalgia rheumatica (PMR) affects mostly subjects who are more than 50 years of age. The prevalence of PMR is highest among subjects from Scandinavian countries and those from northern European origin.

==Risk Factors==
Age, female sex, Scandinavia and northern Europe origin are risk factors for polymyalgia rheumatica. Other risk factors include [[smoking]], [[sun exposure]], [[Infection|infections]], and nulliparity.

== Screening ==
There is insufficient evidence to recommend routine screening for polymyalgia rheumatica.

==Natural History, Complications and Prognosis==
Polymyalgia rheumatica (PMR) affects the quality of life of the patients. The [[pain]] and stiffness in the proximal [[joints]] might lead to sleep disturbance as well as an inability to do regular daily activities such as getting dressed and getting out of a chair. The symptoms begin rapidly and last for weeks. Once the [[steroid]] treatment is initiated, the symptoms resolve rapidly within few days. In fact, the rapid resolution of symptoms with the [[steroid]] therapy reinforces the diagnosis of PMR. The [[steroid]] treatment can be associated with complications such as [[weight gain]] and [[bone fracture]]. Approximately 40 to 50% of subjects experience a relapse of the symptoms. PMR is associated with [[giant cell arteritis]].

==Diagnosis==
===Diagnostic Study of Choice===
The diagnosis of polymyalgia rheumatica (PMR) is mostly clinical and it is supported with specific findings on laboratory tests and [[ultrasound]] of the affected [[joints]]. The European League Against Rheumatism/American College of Rheumatology collaborative initiative developed a provisional classification criteria for PMR. The following criteria are required for the diagnosis of PMR: age more than 50 years, bilateral [[shoulder pain]], and elevated [[C-reactive protein]] (CRP) and/or [[erythrocyte sedimentation rate]] (ESR).

===History and Symptoms===
Polymyalgia rheumatica (PMR) is typically characterized by symmetrical [[pain]] and morning stiffness in the proximal [[Joint|joints]] and [[Limb (anatomy)|limbs]], including the [[neck]], the [[shoulder girdle]], the [[pelvic girdle]], the [[lower back]], and the [[Thigh|thighs]]. In some patients, there is involvement of the distal parts of the body such as peripheral [[synovitis]] or [[arthritis]]. Constitutional symptoms can also be present, and they include [[fever]], [[fatigue]], [[loss of appetite]], and [[weight loss]]. There is an association between PMR and [[giant cell arteritis]] which can present with one or more of the following symptoms that include [[headaches]], scalp tenderness, [[jaw claudication]], [[fever]], or distorted vision.

===Physical Examination===
Physical examination of patients with polymyalgia rheumatica reveals limitation of the active and passive [[range of motion]] of the affected [[joint]]. There is no true [[muscle weakness]]. There are no changes in the [[Joint|joints]]. [[Ophthalmoscope|Ophthalmoscopic]] exams in patients with polymyalgia rheumatica associated with [[Temporal arteritis|giant cell arteritis]] might be abnormal.

===Laboratory Findings===
Polymyalgia rheumatica (PMR) is a clinical diagnosis that is supported by laboratory tests. Elevation of [[C-reactive protein]] (CRP) and/or [[erythrocyte sedimentation rate]] (ESR) is essential for the diagnosis of PMR.

=== Electrocardiogram ===
There are no EKG findings associated with polymyalgia rheumatica.

===X Ray===
There are no x ray findings associated with polymyalgia rheumatica.

=== Echocardiography and Ultrasound ===
There are no echocardiography findings associated with polymyalgia rheumatica. [[Ultrasound]] exam is important for the diagnosis of polymyalgia rheumatica (PMR). It can reveal evidence of [[bursitis]], [[synovitis]] or [[tenosynovitis]] in the affected areas.

===CT Scan===
There are no CT findings associated with polymyalgia rheumatica.

===MRI===
[[MRI]] is used for the assessment of [[bursitis]], [[synovitis]], and [[tenosynovitis]] among patients with polymyalgia rheumatica (PMR). [[MRI]] is more sensitive than [[ultrasonography]] for the evaluation of iliopsoas [[bursitis]] and hip [[synovitis]]. A study has demonstrated that [[MRI]] of the shoulders facilitates the proper diagnosis in patients with the typical proximal symptoms of [[PMR]] with normal [[ESR]] values.

===Other Imaging Findings===
There are no other imaging findings associated with polymyalgia rheumatica.

===Other Diagnostic Studies===
A [[muscle biopsy]] might be performed to differentiate polymyalgia rheumatica (PMR) from other diseases. In addition, [[temporal artery]] biopsy is required when a subject with PMR have symptoms suggestive of [[giant cell arteritis]].

==Treatment==

===Medical Therapy===
The mainstay of treatment of polymyalgia rheumatica (PMR) is low dose [[glucocorticoids]], typically [[prednisone]] or [[prednisolone]]. The starting dose of the [[glucocorticoid]] treatment is 12.5-15 mg daily for 2 to 4 weeks after which the treatment should be slowly tapered. The average duration of the treatment with [[glucocorticoids]] is 1 to 2 years; nevertheless, longer [[corticosteroids]] regimens might be necessary among patients who experience relapse of the symptoms. Prophylaxis for [[osteoporosis]] with [[calcium]] and [[vitamin D]] should be started with the [[steroid]] therapy.

===Surgery===
Surgical intervention is not recommended for the management of polymyalgia rheumatica.

===Primary Prevention===
There are no established measures for the primary prevention of polymyalgia rheumatica.

===Secondary Prevention===
There are no established measures for the secondary prevention of polymyalgia rheumatica.

==External links==
* [http://www.arthritis.ca/types%20of%20arthritis/polymyalgia%20rheumatica/default.asp?s=1 Polymyalgia Rheumatica] - The Arthritis Society
* [http://www.rheumatology.org/public/factsheets/pmr_new2.asp Patient Education - Polymyalgia Rheumatica] - American College of Rheumatology

[[Category:Medicine]]
[[Category:Rheumatology]]
[[Category:Up-To-Date]]
[[Category:Primary care]]

User:Ayesha A. Khan

2020-05-12T17:57:22Z

Ayesha A. Khan: /* Specialties */

[[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com]

Dr. Ayesha Khan, MD is an Internist in Cleveland, OH.

==Contact==
Email: Ayesha.khan@stvincentcharity.com
==Specialties==
Internist, Rheumatology, Nephrology.

==Hospital affiliations==

*Kindred Hospital-Cleveland-Gateway
*Outreach Professional Services Inc
*St. Vincent Charity Medical Center

Polymyositis and dermatomyositis overview

2020-05-11T16:32:48Z

Ayesha A. Khan: /* Pathophysiology */

__NOTOC__
{{Polymyositis and dermatomyositis}}
{{CMG}}; {{AE}} {{SSH}}
==Overview==
In the late 19th century, polymyositis and dermatomyositis were described by different scientists. Polymyositis and dermatomyositis are subtypes of idiopathic [[inflammatory myopathy]]. Patients develop proximal [[muscle weakness]] manifested as difficulty to raise their arms above the [[Shoulder|shoulders]] or from chair, or climbing stairs and progress to distal [[muscle weakness]]. Patients with dermatomyositis suffer from skin lesions in which Gottron's papules and the heliotrope eruption considered as pathognomonic features. The exact pathogenesis of polymyositis and dermatomyositis is not fully understood. However, it is understood that polymyositis and dermatomyositis are the result of [[Autoimmunity|autoimmune]] attack but triggering factors are not well-known. Polymyositis is caused by [[inflammation]] and [[degeneration]] of the [[Muscle|muscles]]. Dermatomyositis is caused by skin [[inflammation]]. In dermatomyositis, activation and deposition of [[complements]] may lead to lysis of endomysial [[Capillary|capillaries]] and muscle [[ischemia]]. [[Gene|Genes]] including [[HLA-DRB1|HLA DRB1*0301]] and [[HLA-DQ|HLA DQA1*0501]] alleles, and [[tumour necrosis factor]] 308A might be associated with development of polymyositis and dermatomyositis, especially in familial cases. Different conditions associated with polymyositis and dermatomyositis include [[Respiratory system|respiratory]] and [[Heart|cardiac]] diseases, other [[Connective tissue disease|connective tissue diseases]], and [[Cancer|malignancies]]. The [[incidence]] of polymyositis and dermatomyositis is approximately 1-5 per 100,000 individuals worldwide. The [[prevalence]] of polymyositis and dermatomyositis is approximately 5-22 per 100,000 individuals worldwide. The 5-year [[survival rate]] for polymyositis is 75% and for dermatomyositis is 63%. The median survival for polymyositis is 11.0 years and that for dermatomyositis is 12.3 years. [[Muscle biopsy]] is the gold standard test for the diagnosis of polymyositis and dermatomyositis. Prognosis of polymyositis and dermatomyositis varies depends on different studies. Prognosis is generally poor, and the overall [[mortality rate]] of patients with polymyositis and dermatomyositis is approximately 22%. Most common cause of death in patients with polymyositis and dermatomyositis include [[Heart|cardiac]] and [[Lung|pulmonary]] complications, [[Infection|infections]], and [[Cancer|cancers]]. Common risk factors in the development of polymyositis and dermatomyositis include environmental factors such as [[infection]], [[Cancer|malignancy]], and drug toxicities from [[statins]] or immune checkpoint inhibitors. Effective measures for prevention of complications include regular follow ups, [[Cancer|malignancy]] screening, [[Lung|pulmonary]] protection, [[lifestyle]] modification, and prevention of [[medication]]-induced complications.

==Historical Perspective==
In the late 19th century, polymyositis and dermatomyositis were described by different scientists. In 1916, Stertz was the first who described the association between dermatomyositis and [[Cancer|malignancy]]. In 1975, Anthony Bohan and James B. Peter were the first physicians who classified polymyositis and dermatomyositis into 5 subtypes which were used for decades. By 1990, multiple myositis-specific autoantibodies (MSA) were discovered and described. These myositis-specific autoantibodies (MSA) targeting different [[Cytoplasm|cytoplasmic]] ribonucleoproteins and they are used by Love et al. to classify polymyositis and dermatomyositis.

==Classification==
Polymyositis and dermatomyositis is one of the subtypes of idiopathic [[inflammatory myopathy]]. The 2017 European League Against Rheumatism and American College of Rheumatology (EULAR/ACR) classified idiopathic [[inflammatory myopathy]] into 6 subtypes including polymyositis, dermatomyositis, inclusion body myositis, immune-mediated necrotizing myopathies (IMNM), amyopathic dermatomyositis, and juvenile dermatomyositis.

==Pathophysiology==
The exact pathogenesis of polymyositis and dermatomyositis is not fully understood. However, it is understood that polymyositis and dermatomyositis are the result of [[Autoimmunity|autoimmune]] attack but triggering factors are not well-known. Polymyositis is caused by [[inflammation]] and [[degeneration]] of the [[Muscle|muscles]]. In polymyositis, [[CD8-positive cytotoxic T cells]] invade [[Skeletal muscle|muscle fibers]] that express [[MHC class I]] antigens which may leads to fiber [[necrosis]] via the [[perforin]] pathway. [[Hypoxemia|Hypoxia]] may reduce [[Phosphocreatine|creatine phosphate]] and [[adenosine triphosphate]] ([[Adenosine triphosphate|ATP]]) levels in [[muscle]] and lead to [[fatigue]] and [[muscle weakness]]. Dermatomyositis is caused by skin [[inflammation]]. In dermatomyositis, activation and deposition of [[complements]] may lead to lysis of endomysial [[Capillary|capillaries]] and muscle [[ischemia]]. [[Gene|Genes]] including [[HLA-DRB1|HLA DRB1*0301]] and [[HLA-DQ|HLA DQA1*0501]] alleles, and [[tumour necrosis factor]] 308A might be associated with development of polymyositis and dermatomyositis, especially in familial cases. Different conditions associated with polymyositis and dermatomyositis include [[Respiratory system|respiratory]] and [[Heart|cardiac]] diseases, other [[Connective [[tissue]] disease[[connective tissue diseases]], and [[Cancer|malignancies]].The [[inflammatory cells]] are predominantly [[B-cells]] (with smaller numbers of [[CD4]]-positive [[T-cells]]) and are found around blood vessels, in the septa between [[muscle]] [[fascicles]], and in [[fibroadipose tissue]] around [[muscle]]. The key pathological change of [[dermatomyositis]] is a [[vasculitis]], which involves [[endomysial]] and [[perimysial capillaries]] and [[arterioles]]. This [[vasculitis]] begins with [[endothelial]] swelling and is followed by [[endothelial]] [[necrosis]] and [[capillary]] loss. [[Tubuloreticular]] [[cytoplasmic]] inclusions (TRIs) are often seen in [[endothelial cells]]. TRIs also occur in [[lupus]] and other [[collagen]] vascular diseases but are absent in [[polymyositis]] and [[inclusion body]] [[myositis]]. The [[vasculitis]] is thought to be caused by circulating [[anti-endothelial antibodies]]. Interaction of these [[antibodies]] with [[vascular antigens]]activates complement, leading to formation of the [[membranolytic attack complex]] (MAC), which destroys [[endothelial cells]].

==Causes==
The cause of polymyositis and dermatomyositis has not been identified.

==Differentiating Polymyositis and Dermatomyositis from Other Diseases==
Polymyositis and Dermatomyositis must be differentiated from other [[Inflammatory myopathy|inflammatory myopathies]] which cause [[muscle weakness]] and systemic symptoms.

==Epidemiology and Demographics==
The [[incidence]] of polymyositis and dermatomyositis is approximately 1-5 per 100,000 individuals worldwide. The [[prevalence]] of polymyositis and dermatomyositis is approximately 5-22 per 100,000 individuals worldwide. The 5-year [[survival rate]] for polymyositis is 75% and for dermatomyositis is 63%. The median survival for polymyositis is 11.0 years and that for dermatomyositis is 12.3 years. Dermatomyositis has a [[Bimodal distribution|bimodal]] pattern, commonly affects both children and adults over 50 years old. Polymyositis commonly affects adults after second decades of their lives and it is rare among children. There is no racial predilection to polymyositis and dermatomyositis. The female to male ratio is approximately 2 to 1. Prevalence of polymyositis and dermatomyositis are lower in young rural men and higher in older urban women. Pharmacologic medical therapies for polymyositis and dermatomyositis include [[Corticosteroid|corticosteroids]] and [[Disease-modifying antirheumatic drug|disease-modifying antirheumatic drugs]] ([[Disease-modifying antirheumatic drug|DMARDs]]).

==Risk Factors==
Common risk factors in the development of polymyositis and dermatomyositis include environmental factors such as [[infection]], [[Cancer|malignancy]], and drug toxicities from [[statins]] or immune checkpoint inhibitors.

==Screening==
There is insufficient evidence to recommend routine screening for polymyositis and dermatomyositis.

==Natural History, Complications, and Prognosis==

The symptoms of polymyositis and dermatomyositis usually develop very slowly and progressively from proximal [[muscle weakness]] manifested as difficulty to raise their arms above the [[Shoulder|shoulders]] or from chair, or climbing stairs to distal [[muscle weakness]]. In dermatomyositis, myositis develops months to years after [[skin]] manifestations. Common complications of polymyositis and dermatomyositis include [[Cancer|malignancy]], [[Heart|cardiac]], [[Lung|pulmonary]], [[Gastrointestinal tract|gastrointestinal]], [[infection]], and [[medication]] related complications. Prognosis of polymyositis and dermatomyositis varies depends on different studies. Prognosis is generally poor, and the overall [[mortality rate]] of patients with polymyositis and dermatomyositis is approximately 22%. Most common cause of death in patients with polymyositis and dermatomyositis include [[Heart|cardiac]] and [[Lung|pulmonary]] complications, [[Infection|infections]], and [[Cancer|cancers]]. Extramuscular organ involvement, older age of diagnosis, male gender, and non-Caucasian race are associated with a particularly poor [[prognosis]] among patients with polymyositis and dermatomyositis. Younger age of diagnosis and quicker therapy initiation are associated with a better prognosis.
==Diagnosis==
===Diagnostic Study of Choice===
[[Muscle biopsy]] is the gold standard test for the diagnosis of polymyositis and dermatomyositis. [[Necrosis]], increase in endomysial and perimysial [[connective tissue]], [[atrophy]] and [[degeneration]] of both type I and II fibers, especially in a perifascicular distribution might be seen in [[muscle biopsy]]. The [[muscle biopsy]] should be performed when a patient presented with symptoms of [[muscle weakness]] and [[skin rash]] or elevated level of [[muscle]] enzymes.

===History and Symptoms===
Polymyositis and dermatomyositis is a multisystem disorder that involves many [[organs]]. The hallmark of polymyositis is symmetric [[muscle weakness]]. The hallmark of dermatomyositis is [[skin]] manifestation. Patients with polymyositis and dermatomyositis may have a positive history of gradual worsening of proximal [[muscle weakness]], past medical history or family history of other [[Autoimmunity|autoimmune diseases]]. Common symptoms include constitutional symptoms, mild [[Myalgia|myalgias]], [[skin]] eruptions, [[Arthralgia|joint pain]], and [[Edema|swelling]]. Less common symptoms of polymyositis and dermatomyositis include [[cough]], [[dyspnea]], [[Aspiration pneumonia|aspiration]], [[dysphagia]], nasal regurgitation, [[Edema|swelling]] of [[Periorbital edema|periorbital]] area, and [[Nail (anatomy)|fingernails]].

===Physical Examination===
Physical examination of patients with polymyositis and dermatomyositis is usually remarkable for [[muscle weakness]], [[hyporeflexia]], [[skin]] lesions, [[Respiratory system|respiratory]] symptoms. The presence of Gottron's papules and the heliotrope eruption on physical examination is pathognomonic of dermatomyositis. [[Muscle atrophy]] in severe, long standing disease might occur.

===Laboratory Findings===
Elevated [[Sarcoplasmic reticulum|sarcoplasmic]] enzymes are consistent with the diagnosis of polymyositis and dermatomyositis which include [[Creatine kinase|creatine phosphokinase]], [[aldolase]], [[Transaminase|transaminases]], [[Lactate dehydrogenase|lactic dehydrogenase]], and [[myoglobin]]. High [[White blood cells|white blood cell]] counts, low [[Lymphocyte|lymphocytes]], and low [[hematocrit]] levels might be detected on [[Complete blood count|CBC]]. Low [[albumin]] levels, high [[Erythrocyte sedimentation rate|ESR]] and high [[Immunoglobulin M|IgM]]<nowiki/>and [[Immunoglobulin G|IgG]] levels could be seen in patients with polymyositis and dermatomyositis. 20 myositis-specific autoantibodies are identified in patients with polymyositis and dermatomyositis. Anti-Jo-1 antibody is the most frequent myositis-specific autoantibodies which causes antisynthetase syndrome. Anti Mi-2 might be seen frequently in patients with dermatomyositis. Anti-SRP antibody is associated with very poor prognosis. Some of these myositis-specific autoantibodies are associated with [[Cancer|malignancy]]. Anti-HMGCR antibody might be elevated in patients with statin-associated necrotizing autoimmune myopathy (SANAM).

===Electrocardiogram===
An [[The electrocardiogram|ECG]] may be helpful in the diagnosis of [[Heart|cardiac]] complications of polymyositis and dermatomyositis. Findings on an [[The electrocardiogram|ECG]] suggestive of [[Heart|cardiac]] involvement include [[Electrical conduction system of the heart|conduction]]<nowiki/>abnormalities, [[Cardiac arrhythmia|arrhythmias]], [[Left atrium|left atrial]] abnormality, and ST-T changes.

===X-ray===
[[X-rays|X-ray]] may be helpful in the diagnosis of polymyositis, dermatomyositis, and their complications, which include dystrophic [[calcification]] in the [[soft tissue]], [[interstitial lung disease]]<nowiki/>and [[Cancer|malignancy]].

===Echocardiography and Ultrasound===
Different [[ultrasound]] modalities might be used to diagnose polymyositis, which include [[Medical ultrasonography|doppler sonography]], contrast-enhanced [[ultrasound]], and sonoelastography. There are no [[echocardiography]] findings associated with polymyositis and dermatomyositis. However, an [[echocardiography]] may be helpful in the diagnosis of [[Heart|cardiac]] complications of polymyositis and dermatomyositis, which include [[Left ventricle|left ventricular]] [[diastolic dysfunction]], [[Hyperdynamic circulation|hyperdynamic]] heart, [[mitral valve prolapse]], and [[endomyocardial fibrosis]].

===CT scan===
There are no [[Computed tomography|CT scan]] findings associated with polymyositis and dermatomyositis. However, a [[Computed tomography|CT scan]] may be helpful in the diagnosis of complications of polymyositis and dermatomyositis, which include [[interstitial lung disease]] or [[Cancer|malignancy]].

===MRI===
[[Magnetic resonance imaging|MRI]] from [[muscles]] may be helpful in the diagnosis of polymyositis and dermatomyositis. Findings on [[Magnetic resonance imaging|MRI]] suggestive of polymyositis and dermatomyositis include areas of high signal intensity reflecting an active disease and muscle [[edema]], fatty infiltration of [[Muscle|muscles]], and muscle [[calcification]].

===Other Imaging Findings===
There are no other imaging findings associated with polymyositis and dermatomyositis.

===Other Diagnostic Studies===
[[Electromyography|Electromyogram]] may be helpful in the diagnosis of polymyositis and dermatomyositis. Findings suggestive of polymyositis and dermatomyositis include delay in electrical signals between the [[Muscle|muscles]] and [[Nerve|nerves]] when they are stimulated, polyphasic, short, small motor-unit potentials, and [[fibrillation]], positive sharp waves, increased insertional irritability. The first sign of improvement in polymyositis and dermatomyositis is the disappearance of [[fibrillation]] potentials. [[Electromyography|Electromyogram]] may also be used to identifying active sites of [[myositis]] for [[biopsy]], follow up, and the treatment efficacy. [[Skin biopsy]] may be used in dermatomyositis, which demonstrates poikiloderma, epidermal [[atrophy]], and liquefaction degeneration of the basal cells. [[Histology|Histochemical staining]] might be helpful to differentiate polymyositis and dermatomyositis from [[Lower motor neuron lesion|lower-motor-neuron diseases]].

==Treatment==
===Medical Therapy===
Pharmacologic medical therapies for polymyositis and dermatomyositis include [[Corticosteroid|corticosteroids]] and [[Disease-modifying antirheumatic drug|disease-modifying antirheumatic drugs]] ([[Disease-modifying antirheumatic drug|DMARDs]]). Patients with polymyositis and dermatomyositis might require long-term treatment. However, if there are no disease flares during the course of the taper, [[glucocorticoids]] could be discontinued at 9 to 12 months. [[Disease-modifying antirheumatic drug|DMARDs]] might be discontinued at 6 months. Patients with recurrent flare of polymyositis and dermatomyositis require higher dose of [[prednisone]], adding or increasing dose of [[methotrexate]] or [[azathioprine]]. [[Rituximab]], IVIg, and [[Mycophenolate sodium|mycophenolate mofetil]] might be used in resistant diseases.

===Surgery===
Surgical intervention is not recommended for the management of polymyositis and dermatomyositis.

===Primary Prevention===
There are no established measures for the primary prevention of polymyositis and dermatomyositis.

===Secondary Prevention===
Effective measures for the secondary prevention of polymyositis and dermatomyositis include regular follow ups, [[Cancer|malignancy]] screening, [[Lung|pulmonary]] protection, [[lifestyle]] <nowiki/>modification, and prevention of [[medication]]-induced complications.

==References==
{{reflist|2}}

Polymyositis and dermatomyositis overview

2020-05-11T16:26:18Z

Ayesha A. Khan: /* Pathophysiology */

__NOTOC__
{{Polymyositis and dermatomyositis}}
{{CMG}}; {{AE}} {{SSH}}
==Overview==
In the late 19th century, polymyositis and dermatomyositis were described by different scientists. Polymyositis and dermatomyositis are subtypes of idiopathic [[inflammatory myopathy]]. Patients develop proximal [[muscle weakness]] manifested as difficulty to raise their arms above the [[Shoulder|shoulders]] or from chair, or climbing stairs and progress to distal [[muscle weakness]]. Patients with dermatomyositis suffer from skin lesions in which Gottron's papules and the heliotrope eruption considered as pathognomonic features. The exact pathogenesis of polymyositis and dermatomyositis is not fully understood. However, it is understood that polymyositis and dermatomyositis are the result of [[Autoimmunity|autoimmune]] attack but triggering factors are not well-known. Polymyositis is caused by [[inflammation]] and [[degeneration]] of the [[Muscle|muscles]]. Dermatomyositis is caused by skin [[inflammation]]. In dermatomyositis, activation and deposition of [[complements]] may lead to lysis of endomysial [[Capillary|capillaries]] and muscle [[ischemia]]. [[Gene|Genes]] including [[HLA-DRB1|HLA DRB1*0301]] and [[HLA-DQ|HLA DQA1*0501]] alleles, and [[tumour necrosis factor]] 308A might be associated with development of polymyositis and dermatomyositis, especially in familial cases. Different conditions associated with polymyositis and dermatomyositis include [[Respiratory system|respiratory]] and [[Heart|cardiac]] diseases, other [[Connective tissue disease|connective tissue diseases]], and [[Cancer|malignancies]]. The [[incidence]] of polymyositis and dermatomyositis is approximately 1-5 per 100,000 individuals worldwide. The [[prevalence]] of polymyositis and dermatomyositis is approximately 5-22 per 100,000 individuals worldwide. The 5-year [[survival rate]] for polymyositis is 75% and for dermatomyositis is 63%. The median survival for polymyositis is 11.0 years and that for dermatomyositis is 12.3 years. [[Muscle biopsy]] is the gold standard test for the diagnosis of polymyositis and dermatomyositis. Prognosis of polymyositis and dermatomyositis varies depends on different studies. Prognosis is generally poor, and the overall [[mortality rate]] of patients with polymyositis and dermatomyositis is approximately 22%. Most common cause of death in patients with polymyositis and dermatomyositis include [[Heart|cardiac]] and [[Lung|pulmonary]] complications, [[Infection|infections]], and [[Cancer|cancers]]. Common risk factors in the development of polymyositis and dermatomyositis include environmental factors such as [[infection]], [[Cancer|malignancy]], and drug toxicities from [[statins]] or immune checkpoint inhibitors. Effective measures for prevention of complications include regular follow ups, [[Cancer|malignancy]] screening, [[Lung|pulmonary]] protection, [[lifestyle]] modification, and prevention of [[medication]]-induced complications.

==Historical Perspective==
In the late 19th century, polymyositis and dermatomyositis were described by different scientists. In 1916, Stertz was the first who described the association between dermatomyositis and [[Cancer|malignancy]]. In 1975, Anthony Bohan and James B. Peter were the first physicians who classified polymyositis and dermatomyositis into 5 subtypes which were used for decades. By 1990, multiple myositis-specific autoantibodies (MSA) were discovered and described. These myositis-specific autoantibodies (MSA) targeting different [[Cytoplasm|cytoplasmic]] ribonucleoproteins and they are used by Love et al. to classify polymyositis and dermatomyositis.

==Classification==
Polymyositis and dermatomyositis is one of the subtypes of idiopathic [[inflammatory myopathy]]. The 2017 European League Against Rheumatism and American College of Rheumatology (EULAR/ACR) classified idiopathic [[inflammatory myopathy]] into 6 subtypes including polymyositis, dermatomyositis, inclusion body myositis, immune-mediated necrotizing myopathies (IMNM), amyopathic dermatomyositis, and juvenile dermatomyositis.

==Pathophysiology==
The exact pathogenesis of polymyositis and dermatomyositis is not fully understood. However, it is understood that polymyositis and dermatomyositis are the result of [[Autoimmunity|autoimmune]] attack but triggering factors are not well-known. Polymyositis is caused by [[inflammation]] and [[degeneration]] of the [[Muscle|muscles]]. In polymyositis, [[CD8-positive cytotoxic T cells]] invade [[Skeletal muscle|muscle fibers]] that express [[MHC class I]] antigens which may leads to fiber [[necrosis]] via the [[perforin]] pathway. [[Hypoxemia|Hypoxia]] may reduce [[Phosphocreatine|creatine phosphate]] and [[adenosine triphosphate]] ([[Adenosine triphosphate|ATP]]) levels in [[muscle]] and lead to [[fatigue]] and [[muscle weakness]]. Dermatomyositis is caused by skin [[inflammation]]. In dermatomyositis, activation and deposition of [[complements]] may lead to lysis of endomysial [[Capillary|capillaries]] and muscle [[ischemia]]. [[Gene|Genes]] including [[HLA-DRB1|HLA DRB1*0301]] and [[HLA-DQ|HLA DQA1*0501]] alleles, and [[tumour necrosis factor]] 308A might be associated with development of polymyositis and dermatomyositis, especially in familial cases. Different conditions associated with polymyositis and dermatomyositis include [[Respiratory system|respiratory]] and [[Heart|cardiac]] diseases, other [[Connective tissue disease|connective tissue diseases]], and [[Cancer|malignancies]].The inflammatory cells are predominantly B-cells (with smaller numbers of CD4-positive T-cells) and are found around blood vessels, in the septa between muscle fascicles, and in fibroadipose tissue around muscle. The key pathological change of dermatomyositis is a vasculitis, which involves endomysial and perimysial capillaries and arterioles. This vasculitis begins with endothelial swelling and is followed by endothelial necrosis and capillary loss. Tubuloreticular cytoplasmic inclusions (TRIs) are often seen in endothelial cells. TRIs also occur in lupus and other collagen vascular diseases but are absent in polymyositis and inclusion body myositis. The vasculitis is thought to be caused by circulating anti-endothelial antibodies. Interaction of these antibodies with vascular antigens activates complement, leading to formation of the membranolytic attack complex (MAC), which destroys endothelial cells.

==Causes==
The cause of polymyositis and dermatomyositis has not been identified.

==Differentiating Polymyositis and Dermatomyositis from Other Diseases==
Polymyositis and Dermatomyositis must be differentiated from other [[Inflammatory myopathy|inflammatory myopathies]] which cause [[muscle weakness]] and systemic symptoms.

==Epidemiology and Demographics==
The [[incidence]] of polymyositis and dermatomyositis is approximately 1-5 per 100,000 individuals worldwide. The [[prevalence]] of polymyositis and dermatomyositis is approximately 5-22 per 100,000 individuals worldwide. The 5-year [[survival rate]] for polymyositis is 75% and for dermatomyositis is 63%. The median survival for polymyositis is 11.0 years and that for dermatomyositis is 12.3 years. Dermatomyositis has a [[Bimodal distribution|bimodal]] pattern, commonly affects both children and adults over 50 years old. Polymyositis commonly affects adults after second decades of their lives and it is rare among children. There is no racial predilection to polymyositis and dermatomyositis. The female to male ratio is approximately 2 to 1. Prevalence of polymyositis and dermatomyositis are lower in young rural men and higher in older urban women. Pharmacologic medical therapies for polymyositis and dermatomyositis include [[Corticosteroid|corticosteroids]] and [[Disease-modifying antirheumatic drug|disease-modifying antirheumatic drugs]] ([[Disease-modifying antirheumatic drug|DMARDs]]).

==Risk Factors==
Common risk factors in the development of polymyositis and dermatomyositis include environmental factors such as [[infection]], [[Cancer|malignancy]], and drug toxicities from [[statins]] or immune checkpoint inhibitors.

==Screening==
There is insufficient evidence to recommend routine screening for polymyositis and dermatomyositis.

==Natural History, Complications, and Prognosis==

The symptoms of polymyositis and dermatomyositis usually develop very slowly and progressively from proximal [[muscle weakness]] manifested as difficulty to raise their arms above the [[Shoulder|shoulders]] or from chair, or climbing stairs to distal [[muscle weakness]]. In dermatomyositis, myositis develops months to years after [[skin]] manifestations. Common complications of polymyositis and dermatomyositis include [[Cancer|malignancy]], [[Heart|cardiac]], [[Lung|pulmonary]], [[Gastrointestinal tract|gastrointestinal]], [[infection]], and [[medication]] related complications. Prognosis of polymyositis and dermatomyositis varies depends on different studies. Prognosis is generally poor, and the overall [[mortality rate]] of patients with polymyositis and dermatomyositis is approximately 22%. Most common cause of death in patients with polymyositis and dermatomyositis include [[Heart|cardiac]] and [[Lung|pulmonary]] complications, [[Infection|infections]], and [[Cancer|cancers]]. Extramuscular organ involvement, older age of diagnosis, male gender, and non-Caucasian race are associated with a particularly poor [[prognosis]] among patients with polymyositis and dermatomyositis. Younger age of diagnosis and quicker therapy initiation are associated with a better prognosis.
==Diagnosis==
===Diagnostic Study of Choice===
[[Muscle biopsy]] is the gold standard test for the diagnosis of polymyositis and dermatomyositis. [[Necrosis]], increase in endomysial and perimysial [[connective tissue]], [[atrophy]] and [[degeneration]] of both type I and II fibers, especially in a perifascicular distribution might be seen in [[muscle biopsy]]. The [[muscle biopsy]] should be performed when a patient presented with symptoms of [[muscle weakness]] and [[skin rash]] or elevated level of [[muscle]] enzymes.

===History and Symptoms===
Polymyositis and dermatomyositis is a multisystem disorder that involves many [[organs]]. The hallmark of polymyositis is symmetric [[muscle weakness]]. The hallmark of dermatomyositis is [[skin]] manifestation. Patients with polymyositis and dermatomyositis may have a positive history of gradual worsening of proximal [[muscle weakness]], past medical history or family history of other [[Autoimmunity|autoimmune diseases]]. Common symptoms include constitutional symptoms, mild [[Myalgia|myalgias]], [[skin]] eruptions, [[Arthralgia|joint pain]], and [[Edema|swelling]]. Less common symptoms of polymyositis and dermatomyositis include [[cough]], [[dyspnea]], [[Aspiration pneumonia|aspiration]], [[dysphagia]], nasal regurgitation, [[Edema|swelling]] of [[Periorbital edema|periorbital]] area, and [[Nail (anatomy)|fingernails]].

===Physical Examination===
Physical examination of patients with polymyositis and dermatomyositis is usually remarkable for [[muscle weakness]], [[hyporeflexia]], [[skin]] lesions, [[Respiratory system|respiratory]] symptoms. The presence of Gottron's papules and the heliotrope eruption on physical examination is pathognomonic of dermatomyositis. [[Muscle atrophy]] in severe, long standing disease might occur.

===Laboratory Findings===
Elevated [[Sarcoplasmic reticulum|sarcoplasmic]] enzymes are consistent with the diagnosis of polymyositis and dermatomyositis which include [[Creatine kinase|creatine phosphokinase]], [[aldolase]], [[Transaminase|transaminases]], [[Lactate dehydrogenase|lactic dehydrogenase]], and [[myoglobin]]. High [[White blood cells|white blood cell]] counts, low [[Lymphocyte|lymphocytes]], and low [[hematocrit]] levels might be detected on [[Complete blood count|CBC]]. Low [[albumin]] levels, high [[Erythrocyte sedimentation rate|ESR]] and high [[Immunoglobulin M|IgM]]<nowiki/>and [[Immunoglobulin G|IgG]] levels could be seen in patients with polymyositis and dermatomyositis. 20 myositis-specific autoantibodies are identified in patients with polymyositis and dermatomyositis. Anti-Jo-1 antibody is the most frequent myositis-specific autoantibodies which causes antisynthetase syndrome. Anti Mi-2 might be seen frequently in patients with dermatomyositis. Anti-SRP antibody is associated with very poor prognosis. Some of these myositis-specific autoantibodies are associated with [[Cancer|malignancy]]. Anti-HMGCR antibody might be elevated in patients with statin-associated necrotizing autoimmune myopathy (SANAM).

===Electrocardiogram===
An [[The electrocardiogram|ECG]] may be helpful in the diagnosis of [[Heart|cardiac]] complications of polymyositis and dermatomyositis. Findings on an [[The electrocardiogram|ECG]] suggestive of [[Heart|cardiac]] involvement include [[Electrical conduction system of the heart|conduction]]<nowiki/>abnormalities, [[Cardiac arrhythmia|arrhythmias]], [[Left atrium|left atrial]] abnormality, and ST-T changes.

===X-ray===
[[X-rays|X-ray]] may be helpful in the diagnosis of polymyositis, dermatomyositis, and their complications, which include dystrophic [[calcification]] in the [[soft tissue]], [[interstitial lung disease]]<nowiki/>and [[Cancer|malignancy]].

===Echocardiography and Ultrasound===
Different [[ultrasound]] modalities might be used to diagnose polymyositis, which include [[Medical ultrasonography|doppler sonography]], contrast-enhanced [[ultrasound]], and sonoelastography. There are no [[echocardiography]] findings associated with polymyositis and dermatomyositis. However, an [[echocardiography]] may be helpful in the diagnosis of [[Heart|cardiac]] complications of polymyositis and dermatomyositis, which include [[Left ventricle|left ventricular]] [[diastolic dysfunction]], [[Hyperdynamic circulation|hyperdynamic]] heart, [[mitral valve prolapse]], and [[endomyocardial fibrosis]].

===CT scan===
There are no [[Computed tomography|CT scan]] findings associated with polymyositis and dermatomyositis. However, a [[Computed tomography|CT scan]] may be helpful in the diagnosis of complications of polymyositis and dermatomyositis, which include [[interstitial lung disease]] or [[Cancer|malignancy]].

===MRI===
[[Magnetic resonance imaging|MRI]] from [[muscles]] may be helpful in the diagnosis of polymyositis and dermatomyositis. Findings on [[Magnetic resonance imaging|MRI]] suggestive of polymyositis and dermatomyositis include areas of high signal intensity reflecting an active disease and muscle [[edema]], fatty infiltration of [[Muscle|muscles]], and muscle [[calcification]].

===Other Imaging Findings===
There are no other imaging findings associated with polymyositis and dermatomyositis.

===Other Diagnostic Studies===
[[Electromyography|Electromyogram]] may be helpful in the diagnosis of polymyositis and dermatomyositis. Findings suggestive of polymyositis and dermatomyositis include delay in electrical signals between the [[Muscle|muscles]] and [[Nerve|nerves]] when they are stimulated, polyphasic, short, small motor-unit potentials, and [[fibrillation]], positive sharp waves, increased insertional irritability. The first sign of improvement in polymyositis and dermatomyositis is the disappearance of [[fibrillation]] potentials. [[Electromyography|Electromyogram]] may also be used to identifying active sites of [[myositis]] for [[biopsy]], follow up, and the treatment efficacy. [[Skin biopsy]] may be used in dermatomyositis, which demonstrates poikiloderma, epidermal [[atrophy]], and liquefaction degeneration of the basal cells. [[Histology|Histochemical staining]] might be helpful to differentiate polymyositis and dermatomyositis from [[Lower motor neuron lesion|lower-motor-neuron diseases]].

==Treatment==
===Medical Therapy===
Pharmacologic medical therapies for polymyositis and dermatomyositis include [[Corticosteroid|corticosteroids]] and [[Disease-modifying antirheumatic drug|disease-modifying antirheumatic drugs]] ([[Disease-modifying antirheumatic drug|DMARDs]]). Patients with polymyositis and dermatomyositis might require long-term treatment. However, if there are no disease flares during the course of the taper, [[glucocorticoids]] could be discontinued at 9 to 12 months. [[Disease-modifying antirheumatic drug|DMARDs]] might be discontinued at 6 months. Patients with recurrent flare of polymyositis and dermatomyositis require higher dose of [[prednisone]], adding or increasing dose of [[methotrexate]] or [[azathioprine]]. [[Rituximab]], IVIg, and [[Mycophenolate sodium|mycophenolate mofetil]] might be used in resistant diseases.

===Surgery===
Surgical intervention is not recommended for the management of polymyositis and dermatomyositis.

===Primary Prevention===
There are no established measures for the primary prevention of polymyositis and dermatomyositis.

===Secondary Prevention===
Effective measures for the secondary prevention of polymyositis and dermatomyositis include regular follow ups, [[Cancer|malignancy]] screening, [[Lung|pulmonary]] protection, [[lifestyle]] <nowiki/>modification, and prevention of [[medication]]-induced complications.

==References==
{{reflist|2}}

Polymyositis and dermatomyositis overview

2020-05-11T16:21:36Z

Ayesha A. Khan:

__NOTOC__
{{Polymyositis and dermatomyositis}}
{{CMG}}; {{AE}} {{SSH}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com]

==Overview==
In the late 19th century, polymyositis and dermatomyositis were described by different scientists. Polymyositis and dermatomyositis are subtypes of idiopathic [[inflammatory myopathy]]. Patients develop proximal [[muscle weakness]] manifested as difficulty to raise their arms above the [[Shoulder|shoulders]] or from chair, or climbing stairs and progress to distal [[muscle weakness]]. Patients with dermatomyositis suffer from skin lesions in which Gottron's papules and the heliotrope eruption considered as pathognomonic features. The exact pathogenesis of polymyositis and dermatomyositis is not fully understood. However, it is understood that polymyositis and dermatomyositis are the result of [[Autoimmunity|autoimmune]] attack but triggering factors are not well-known. Polymyositis is caused by [[inflammation]] and [[degeneration]] of the [[Muscle|muscles]]. Dermatomyositis is caused by skin [[inflammation]]. In dermatomyositis, activation and deposition of [[complements]] may lead to lysis of endomysial [[Capillary|capillaries]] and muscle [[ischemia]]. [[Gene|Genes]] including [[HLA-DRB1|HLA DRB1*0301]] and [[HLA-DQ|HLA DQA1*0501]] alleles, and [[tumour necrosis factor]] 308A might be associated with development of polymyositis and dermatomyositis, especially in familial cases. Different conditions associated with polymyositis and dermatomyositis include [[Respiratory system|respiratory]] and [[Heart|cardiac]] diseases, other [[Connective tissue disease|connective tissue diseases]], and [[Cancer|malignancies]]. The [[incidence]] of polymyositis and dermatomyositis is approximately 1-5 per 100,000 individuals worldwide. The [[prevalence]] of polymyositis and dermatomyositis is approximately 5-22 per 100,000 individuals worldwide. The 5-year [[survival rate]] for polymyositis is 75% and for dermatomyositis is 63%. The median survival for polymyositis is 11.0 years and that for dermatomyositis is 12.3 years. [[Muscle biopsy]] is the gold standard test for the diagnosis of polymyositis and dermatomyositis. Prognosis of polymyositis and dermatomyositis varies depends on different studies. Prognosis is generally poor, and the overall [[mortality rate]] of patients with polymyositis and dermatomyositis is approximately 22%. Most common cause of death in patients with polymyositis and dermatomyositis include [[Heart|cardiac]] and [[Lung|pulmonary]] complications, [[Infection|infections]], and [[Cancer|cancers]]. Common risk factors in the development of polymyositis and dermatomyositis include environmental factors such as [[infection]], [[Cancer|malignancy]], and drug toxicities from [[statins]] or immune checkpoint inhibitors. Effective measures for prevention of complications include regular follow ups, [[Cancer|malignancy]] screening, [[Lung|pulmonary]] protection, [[lifestyle]] modification, and prevention of [[medication]]-induced complications.

==Historical Perspective==
In the late 19th century, polymyositis and dermatomyositis were described by different scientists. In 1916, Stertz was the first who described the association between dermatomyositis and [[Cancer|malignancy]]. In 1975, Anthony Bohan and James B. Peter were the first physicians who classified polymyositis and dermatomyositis into 5 subtypes which were used for decades. By 1990, multiple myositis-specific autoantibodies (MSA) were discovered and described. These myositis-specific autoantibodies (MSA) targeting different [[Cytoplasm|cytoplasmic]] ribonucleoproteins and they are used by Love et al. to classify polymyositis and dermatomyositis.

==Classification==
Polymyositis and dermatomyositis is one of the subtypes of idiopathic [[inflammatory myopathy]]. The 2017 European League Against Rheumatism and American College of Rheumatology (EULAR/ACR) classified idiopathic [[inflammatory myopathy]] into 6 subtypes including polymyositis, dermatomyositis, inclusion body myositis, immune-mediated necrotizing myopathies (IMNM), amyopathic dermatomyositis, and juvenile dermatomyositis.

==Pathophysiology==
The exact pathogenesis of polymyositis and dermatomyositis is not fully understood. However, it is understood that polymyositis and dermatomyositis are the result of [[Autoimmunity|autoimmune]] attack but triggering factors are not well-known. Polymyositis is caused by [[inflammation]] and [[degeneration]] of the [[Muscle|muscles]]. In polymyositis, [[CD8-positive cytotoxic T cells]] invade [[Skeletal muscle|muscle fibers]] that express [[MHC class I]] antigens which may leads to fiber [[necrosis]] via the [[perforin]] pathway. [[Hypoxemia|Hypoxia]] may reduce [[Phosphocreatine|creatine phosphate]] and [[adenosine triphosphate]] ([[Adenosine triphosphate|ATP]]) levels in [[muscle]] and lead to [[fatigue]] and [[muscle weakness]]. Dermatomyositis is caused by skin [[inflammation]]. In dermatomyositis, activation and deposition of [[complements]] may lead to lysis of endomysial [[Capillary|capillaries]] and muscle [[ischemia]]. [[Gene|Genes]] including [[HLA-DRB1|HLA DRB1*0301]] and [[HLA-DQ|HLA DQA1*0501]] alleles, and [[tumour necrosis factor]] 308A might be associated with development of polymyositis and dermatomyositis, especially in familial cases. Different conditions associated with polymyositis and dermatomyositis include [[Respiratory system|respiratory]] and [[Heart|cardiac]] diseases, other [[Connective [[tissue]] disease[[connective tissue diseases]], and [[Cancer|malignancies]].The [[inflammatory cells]] are predominantly [[B-cells]] (with smaller numbers of [[CD4]]-positive [[T-cells]]) and are found around blood vessels, in the septa between [[muscle]] [[fascicles]], and in [[fibroadipose tissue]] around [[muscle]]. The key pathological change of [[dermatomyositis]] is a [[vasculitis]], which involves [[endomysial]] and [[perimysial capillaries]] and [[arterioles]]. This [[vasculitis]] begins with [[endothelial]] swelling and is followed by [[endothelial]] [[necrosis]] and [[capillary]] loss. [[Tubuloreticular]] [[cytoplasmic]] inclusions (TRIs) are often seen in [[endothelial cells]]. TRIs also occur in [[lupus]] and other [[collagen]] vascular diseases but are absent in [[polymyositis]] and [[inclusion body]] [[myositis]]. The [[vasculitis]] is thought to be caused by circulating [[anti-endothelial antibodies]]. Interaction of these [[antibodies]] with [[vascular antigens]]activates complement, leading to formation of the [[membranolytic attack complex]] (MAC), which destroys [[endothelial cells]].

==Causes==
The cause of polymyositis and dermatomyositis has not been identified.

==Differentiating Polymyositis and Dermatomyositis from Other Diseases==
Polymyositis and Dermatomyositis must be differentiated from other [[Inflammatory myopathy|inflammatory myopathies]] which cause [[muscle weakness]] and systemic symptoms.

==Epidemiology and Demographics==
The [[incidence]] of polymyositis and dermatomyositis is approximately 1-5 per 100,000 individuals worldwide. The [[prevalence]] of polymyositis and dermatomyositis is approximately 5-22 per 100,000 individuals worldwide. The 5-year [[survival rate]] for polymyositis is 75% and for dermatomyositis is 63%. The median survival for polymyositis is 11.0 years and that for dermatomyositis is 12.3 years. Dermatomyositis has a [[Bimodal distribution|bimodal]] pattern, commonly affects both children and adults over 50 years old. Polymyositis commonly affects adults after second decades of their lives and it is rare among children. There is no racial predilection to polymyositis and dermatomyositis. The female to male ratio is approximately 2 to 1. Prevalence of polymyositis and dermatomyositis are lower in young rural men and higher in older urban women. Pharmacologic medical therapies for polymyositis and dermatomyositis include [[Corticosteroid|corticosteroids]] and [[Disease-modifying antirheumatic drug|disease-modifying antirheumatic drugs]] ([[Disease-modifying antirheumatic drug|DMARDs]]).

==Risk Factors==
Common risk factors in the development of polymyositis and dermatomyositis include environmental factors such as [[infection]], [[Cancer|malignancy]], and drug toxicities from [[statins]] or immune checkpoint inhibitors.

==Screening==
There is insufficient evidence to recommend routine screening for polymyositis and dermatomyositis.

==Natural History, Complications, and Prognosis==

The symptoms of polymyositis and dermatomyositis usually develop very slowly and progressively from proximal [[muscle weakness]] manifested as difficulty to raise their arms above the [[Shoulder|shoulders]] or from chair, or climbing stairs to distal [[muscle weakness]]. In dermatomyositis, myositis develops months to years after [[skin]] manifestations. Common complications of polymyositis and dermatomyositis include [[Cancer|malignancy]], [[Heart|cardiac]], [[Lung|pulmonary]], [[Gastrointestinal tract|gastrointestinal]], [[infection]], and [[medication]] related complications. Prognosis of polymyositis and dermatomyositis varies depends on different studies. Prognosis is generally poor, and the overall [[mortality rate]] of patients with polymyositis and dermatomyositis is approximately 22%. Most common cause of death in patients with polymyositis and dermatomyositis include [[Heart|cardiac]] and [[Lung|pulmonary]] complications, [[Infection|infections]], and [[Cancer|cancers]]. Extramuscular organ involvement, older age of diagnosis, male gender, and non-Caucasian race are associated with a particularly poor [[prognosis]] among patients with polymyositis and dermatomyositis. Younger age of diagnosis and quicker therapy initiation are associated with a better prognosis.
==Diagnosis==
===Diagnostic Study of Choice===
[[Muscle biopsy]] is the gold standard test for the diagnosis of polymyositis and dermatomyositis. [[Necrosis]], increase in endomysial and perimysial [[connective tissue]], [[atrophy]] and [[degeneration]] of both type I and II fibers, especially in a perifascicular distribution might be seen in [[muscle biopsy]]. The [[muscle biopsy]] should be performed when a patient presented with symptoms of [[muscle weakness]] and [[skin rash]] or elevated level of [[muscle]] enzymes.

===History and Symptoms===
Polymyositis and dermatomyositis is a multisystem disorder that involves many [[organs]]. The hallmark of polymyositis is symmetric [[muscle weakness]]. The hallmark of dermatomyositis is [[skin]] manifestation. Patients with polymyositis and dermatomyositis may have a positive history of gradual worsening of proximal [[muscle weakness]], past medical history or family history of other [[Autoimmunity|autoimmune diseases]]. Common symptoms include constitutional symptoms, mild [[Myalgia|myalgias]], [[skin]] eruptions, [[Arthralgia|joint pain]], and [[Edema|swelling]]. Less common symptoms of polymyositis and dermatomyositis include [[cough]], [[dyspnea]], [[Aspiration pneumonia|aspiration]], [[dysphagia]], nasal regurgitation, [[Edema|swelling]] of [[Periorbital edema|periorbital]] area, and [[Nail (anatomy)|fingernails]].

===Physical Examination===
Physical examination of patients with polymyositis and dermatomyositis is usually remarkable for [[muscle weakness]], [[hyporeflexia]], [[skin]] lesions, [[Respiratory system|respiratory]] symptoms. The presence of Gottron's papules and the heliotrope eruption on physical examination is pathognomonic of dermatomyositis. [[Muscle atrophy]] in severe, long standing disease might occur.

===Laboratory Findings===
Elevated [[Sarcoplasmic reticulum|sarcoplasmic]] enzymes are consistent with the diagnosis of polymyositis and dermatomyositis which include [[Creatine kinase|creatine phosphokinase]], [[aldolase]], [[Transaminase|transaminases]], [[Lactate dehydrogenase|lactic dehydrogenase]], and [[myoglobin]]. High [[White blood cells|white blood cell]] counts, low [[Lymphocyte|lymphocytes]], and low [[hematocrit]] levels might be detected on [[Complete blood count|CBC]]. Low [[albumin]] levels, high [[Erythrocyte sedimentation rate|ESR]] and high [[Immunoglobulin M|IgM]]<nowiki/>and [[Immunoglobulin G|IgG]] levels could be seen in patients with polymyositis and dermatomyositis. 20 myositis-specific autoantibodies are identified in patients with polymyositis and dermatomyositis. Anti-Jo-1 antibody is the most frequent myositis-specific autoantibodies which causes antisynthetase syndrome. Anti Mi-2 might be seen frequently in patients with dermatomyositis. Anti-SRP antibody is associated with very poor prognosis. Some of these myositis-specific autoantibodies are associated with [[Cancer|malignancy]]. Anti-HMGCR antibody might be elevated in patients with statin-associated necrotizing autoimmune myopathy (SANAM).

===Electrocardiogram===
An [[The electrocardiogram|ECG]] may be helpful in the diagnosis of [[Heart|cardiac]] complications of polymyositis and dermatomyositis. Findings on an [[The electrocardiogram|ECG]] suggestive of [[Heart|cardiac]] involvement include [[Electrical conduction system of the heart|conduction]]<nowiki/>abnormalities, [[Cardiac arrhythmia|arrhythmias]], [[Left atrium|left atrial]] abnormality, and ST-T changes.

===X-ray===
[[X-rays|X-ray]] may be helpful in the diagnosis of polymyositis, dermatomyositis, and their complications, which include dystrophic [[calcification]] in the [[soft tissue]], [[interstitial lung disease]]<nowiki/>and [[Cancer|malignancy]].

===Echocardiography and Ultrasound===
Different [[ultrasound]] modalities might be used to diagnose polymyositis, which include [[Medical ultrasonography|doppler sonography]], contrast-enhanced [[ultrasound]], and sonoelastography. There are no [[echocardiography]] findings associated with polymyositis and dermatomyositis. However, an [[echocardiography]] may be helpful in the diagnosis of [[Heart|cardiac]] complications of polymyositis and dermatomyositis, which include [[Left ventricle|left ventricular]] [[diastolic dysfunction]], [[Hyperdynamic circulation|hyperdynamic]] heart, [[mitral valve prolapse]], and [[endomyocardial fibrosis]].

===CT scan===
There are no [[Computed tomography|CT scan]] findings associated with polymyositis and dermatomyositis. However, a [[Computed tomography|CT scan]] may be helpful in the diagnosis of complications of polymyositis and dermatomyositis, which include [[interstitial lung disease]] or [[Cancer|malignancy]].

===MRI===
[[Magnetic resonance imaging|MRI]] from [[muscles]] may be helpful in the diagnosis of polymyositis and dermatomyositis. Findings on [[Magnetic resonance imaging|MRI]] suggestive of polymyositis and dermatomyositis include areas of high signal intensity reflecting an active disease and muscle [[edema]], fatty infiltration of [[Muscle|muscles]], and muscle [[calcification]].

===Other Imaging Findings===
There are no other imaging findings associated with polymyositis and dermatomyositis.

===Other Diagnostic Studies===
[[Electromyography|Electromyogram]] may be helpful in the diagnosis of polymyositis and dermatomyositis. Findings suggestive of polymyositis and dermatomyositis include delay in electrical signals between the [[Muscle|muscles]] and [[Nerve|nerves]] when they are stimulated, polyphasic, short, small motor-unit potentials, and [[fibrillation]], positive sharp waves, increased insertional irritability. The first sign of improvement in polymyositis and dermatomyositis is the disappearance of [[fibrillation]] potentials. [[Electromyography|Electromyogram]] may also be used to identifying active sites of [[myositis]] for [[biopsy]], follow up, and the treatment efficacy. [[Skin biopsy]] may be used in dermatomyositis, which demonstrates poikiloderma, epidermal [[atrophy]], and liquefaction degeneration of the basal cells. [[Histology|Histochemical staining]] might be helpful to differentiate polymyositis and dermatomyositis from [[Lower motor neuron lesion|lower-motor-neuron diseases]].

==Treatment==
===Medical Therapy===
Pharmacologic medical therapies for polymyositis and dermatomyositis include [[Corticosteroid|corticosteroids]] and [[Disease-modifying antirheumatic drug|disease-modifying antirheumatic drugs]] ([[Disease-modifying antirheumatic drug|DMARDs]]). Patients with polymyositis and dermatomyositis might require long-term treatment. However, if there are no disease flares during the course of the taper, [[glucocorticoids]] could be discontinued at 9 to 12 months. [[Disease-modifying antirheumatic drug|DMARDs]] might be discontinued at 6 months. Patients with recurrent flare of polymyositis and dermatomyositis require higher dose of [[prednisone]], adding or increasing dose of [[methotrexate]] or [[azathioprine]]. [[Rituximab]], IVIg, and [[Mycophenolate sodium|mycophenolate mofetil]] might be used in resistant diseases.

===Surgery===
Surgical intervention is not recommended for the management of polymyositis and dermatomyositis.

===Primary Prevention===
There are no established measures for the primary prevention of polymyositis and dermatomyositis.

===Secondary Prevention===
Effective measures for the secondary prevention of polymyositis and dermatomyositis include regular follow ups, [[Cancer|malignancy]] screening, [[Lung|pulmonary]] protection, [[lifestyle]] <nowiki/>modification, and prevention of [[medication]]-induced complications.

==References==
{{reflist|2}}

Rheumatology

2020-05-10T22:06:01Z

Ayesha A. Khan:

{{SI}}

{{CMG}} {{AE}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com]

==Overview==
'''[[Rheumatology]]''', a subspecialty of [[internal medicine]] and [[pediatrics]] is devoted to the [[diagnosis]] and therapy of '''[[rheumatic]] diseases'''. The term originates from the Greek ''rheuma'', meaning "that which flows as a river or stream" and the suffix ''-ology'', meaning "the study of". Rheumatologists mainly deal with problems involving the joints and the allied conditions of connective tissue.

[[Rheumatology]] is a rapidly evolving specialty; new scientific discoveries related to this specialty are largely related to better understanding of [[immunology]] of these disorders. [[Pathogenesis]] of major rheumatological disorders is now described as auto immune disorders. [[Immunology]] explains [[pathogenesis]] and the characteristics of [[rheumatological]] disorders, and the new treatment modalities are also based on [[immunology]], better understanding of [[genetic]] basis of rheumatological disorders makes [[rheumatology]] a specialty rapidly developing as a specialty based on new scientific discoveries. New treatment modalities are based on scientific research on [[immunology]], [[cytokines]], [[T lymphocytes]], [[B lymphocytes]] and the future therapies may be directed more towards [[gene]] therapy as well. Currently, practice of [[rheumatology]] is largely based on clinical research, evidence based medical treatment of [[rheumatological]] disorders has helped patients with [[rheumatism]] lead a near normal life without any disabilities. Those clinicians specialized on this [[specialty]] are called [[rheumatologists]].

== Rheumatism ==

'''[[Rheumatism]]''' is a non-specific term used to describe any painful disorder affecting the loco-motor system including [[joints]], [[muscles]], [[connective tissues]], [[soft tissues]] around the [[joints]] and [[bones]]. The term rheumatism is also used to describe rheumatic fever affecting [[heart valves]]. However, the medical profession use specific terms to describe rheumatological disorders such as [[rheumatoid arthritis]], [[ankylosing spondylitis]], [[gout]] and [[systemic lupus erythematosus]] and so on in the medical literature.

[[Rheumatology]] is now emerging as an important clinical specialty all over the world, along with well organized post graduate training programs organized for the postgraduate trainees in this field. The term describing clinicians as "[[rheumatologists]]" is now a well established term in the medical community, even though it is not well described in language dictionaries. Rheumatologists all over the world are now capable of treating most of the chronic rheumatological disorders with a much better outcome for the patients that is with the discovery of new disease modifying agents called biologics which is now a well established form of [[treatment]] for the patients suffering with chronic and disabling joint disorders. Large proportion of patients with [[rheumatoid arthritis]], up to seventy percent according to some studies can now be cured with the introduction and wide spread use of biologic treatment for the treatment of arthritic disorders since the beginning of twenty first century.

== Rheumatologist ==
'''Rheumatologist.''' (Consultant Rheumatologist.)

Rheumatologist is a clinician specialized in the field of medical subspecialty called rheumatology and hold either a Doctor of Osteopathy degree (D.O.) or Doctor of Medicine Degree (M.D.). Training in this field requires four years undergraduate school, 4 years of medical school, and then postgraduate training. Rheumatologists are internists, physicians or pediatricians who are qualified by additional postgraduate training and experience in the diagnosis and treatment of arthritis and other diseases of the joints, muscles and bones. Many rheumatologists also conduct research to determine the cause and better treatments for these disabling and sometimes fatal diseases. Treatment modalities are also based on scientific research, currently, practice of rheumatology is largely evidence based. Those clinicians specialized on this specialty are called rheumatologists.

Rheumatologists treat arthritis, certain autoimmune diseases, musculoskeletal pain disorders and osteoporosis. There are more than 200 types of these diseases, including rheumatoid arthritis, osteoarthritis, gout, lupus, back pain, osteoporosis, fibromyalgia and [[tendinitis]]. Some of these are very serious diseases that can be difficult to diagnose and treat. They treat soft tissue problems related to musculoskeletal system sports related soft tissue disorders and the specialty is also interrelated with physiotherapy, physical medicine and rehabilitation of disabled patients. Patient education programmes and occupational therapy is also goes hand in hand with this specialty.

There are many '''international organizations representing Rheumatologists''' all over the world. American College of Rheumatology( ACR), the European League Against Rheumatism (EULAR), Asia Pacific League of Associations for Rheumatology(APLAR), International League of Associations for Rheumatology (ILAR) are the main international organizations established and organizing many activities related to this specialty, these organizations strive to propagate and consolidate Rheumatology endeavors internationally , furthermore, there are Associations and Colleges of Rheumatology representing Rheumatologists from each and every nation scattered throughout the world which represent the above mentioned organizations from each nation. Rheumatologists are physicians specialized in rheumatic diseases.
For example, there are approximately 480 consultant rheumatologists in the UK. Rheumatologists are increasing in numbers in all countries, as there is an increasing demand for specialists on this field with an increasing population of ageing patients who need specialized treatment.

== Diseases ==
Diseases diagnosed or managed by the rheumatologist include:

*[[Rheumatoid arthritis]]
*[[lupus erythematosus]]
*[[Sjögren's syndrome]]
*[[scleroderma]] (systemic sclerosis)
*[[dermatomyositis]]
*[[polychondritis]]
*[[polymyositis]]
*[[polymyalgia rheumatica]]
*[[osteoarthritis]]
*[[septic arthritis]]
*[[fibromyalgia]]
*[[sarcoidosis]]
*[[gout]], [[pseudogout]]
*[[spondyloarthropathy|spondyloarthropathies]]
**[[ankylosing spondylitis]]
**[[reactive arthritis]]
**[[psoriatic arthropathy]]
**[[enteropathic spondylitis]]
**[[reactive arthropathy]]
*[[vasculitis]]
**[[polyarteritis nodosa]]
**[[Henoch-Schönlein purpura]]
**[[serum sickness]]
**[[Wegener's granulomatosis]]
**[[giant cell arteritis]]
**[[temporal arteritis]]
**[[Takayasu's arteritis]]
**[[Behçet's syndrome]]
**[[Kawasaki's disease]] (mucocutaneous lymph node syndrome)
**[[Buerger's disease]] ([[thromboangiitis obliterans]])
*Juvenile Idiopathic Arthritis (JIA)



== Diagnosis ==
Apart from an extensive medical history, there are useful methods of diagnosis both performed easy enough in a [[physical examination]] and, on the other hand, more complicated ones, often requiring a rheumatologist or other specialised physicians.

===Physical examination===
Following are examples of methods of diagnosis able to be performed in a normal physical examination.
* [[Schober's test]] tests the flexion of the [[lower back]].

===Specialised===
* [[Medical laboratory|Laboratory]] [[blood test|tests]] (e.g. [[erythrocyte sedimentation rate]], [[rheumatoid factor]])
* [[X-ray]]s of affected joints and other imaging methods
* [[Cytology]] and [[clinical chemistry|chemical pathology]] of fluid aspirated from affected joints (e.g. to differentiate between [[septic arthritis]] and [[gout]])

== Treatment ==
Most rheumatic diseases are treated with [[analgesic]]s, [[NSAID]]s (Non-Steroid Anti-Inflammatory Drugs), [[steroid]]s (in serious cases), [[DMARD]]s (Disease-Modifying Anti-Rheumatic Drugs), [[monoclonal antibody|monoclonal antibodies]], such as [[infliximab]] and [[adalimumab]], and the soluble TNF receptor [[etanercept]].

[[Physiotherapy]] is vital in the treatment of many rheumatological disorders. [[Occupational therapy]] can help patients finding alternative ways for common movements which would otherwise be restricted by their disease.

== Scientific research ==
Recently, a large body of scientific research deals with the background of [[autoimmune disease]], the cause of many rheumatic disorders. Also, the field of [[osteoimmunology]] has emerged to further examine the interactions between the immune system, joints and bones. Epidemiological studies and medication trials are also being conducted.

==External links==
*[http://www.institutferran.org IFR: Institut Ferran de Reumatologia].
*[http://www.aplar.org/index.html APLAR]

{{Medicine}}

[[Category:Rheumatology|*]]
[[Category:Subjects taught in medical school]]

[[de:Rheumatologie]]
[[et:Reumatoloogia]]
[[es:Reumatología]]
[[eu:Erreumatologia]]
[[fr:Rhumatologie]]
[[io:Reumatologio]]
[[id:Rematologi]]
[[it:Reumatologia]]
[[he:ראומטולוגיה]]
[[ja:リウマチ学]]
[[no:Revmatologi]]
[[pl:Reumatologia]]
[[pt:Reumatologia]]
[[ru:Ревматология]]
[[sv:Reumatologi]]
[[tr:Romatoloji]]

{{WH}}
{{WS}}

Scleroderma overview

2020-05-10T22:04:49Z

Ayesha A. Khan:

__NOTOC__
{{Scleroderma}}
{{CMG}}; {{AE}} {{MKA}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com]

==Overview==
Scleroderma is an [[autoimmune]] [[connective tissue disease]]. The hallmark of the disease is the presence of [[autoantibodies]] against various [[cellular]] [[antigens]], which results in small [[vessel]] [[vasculopathy]], excessive [[collagen]] deposition and [[fibrosis]] of [[skin]] and internal organs. [[Growth factors]] and [[cytokines]] play an important role in the underlying [[pathogenesis]]. The word scleroderma is greek in origin and translates in to hard [[skin]], describing the [[skin]] [[fibrosis]]. Scleroderma is classified into 2 subtype, limited cutaneous scleroderma and diffuse [[cutaneous]] scleroderma. [[CREST syndrome]] is a variant of limited [[cutaneous]] scleroderma. Choctaw native Americans have a much higher [[prevalence]] of scleroderma than the general population. Scleroderma must be differentiated from other diseases that cause [[skin]] thickening, [[sclerodactyly]], [[edema]] and symptoms of [[gastroesophageal reflux disease]]. Common risk factors for scleroderma that have been described in literature are exposure to [[silica]], chlorinated and [[aromatic]] solvents and welding [[fumes]]. Since there is a possibility of internal organ involvement, screening for fatal complications such as scleroderma [[renal]] crisis, [[pulmonary arterial hypertension]], and [[cardiovascular]] involvement must be carried out in patients with scleroderma. Scleroderma is mainly diagnosed based on clinical presentation and the new [[American College of Rheumatology|American College of Rheumatology (ACR)]]/ European League Against Rheumatism (EULAR) classification criteria has been published. The new criteria is more sensitive and is better for detecting disease earlier, so that the fatal [[renal]] and [[pulmonary]] complications can be screened for and prevented. Nailfold video capillaroscopy is the diagnostic study of choice to confirm [[Raynaud's phenomenon]] and scleroderma [[microangiopathy]]. The mainstay of treatment for scleroderma is medical therapy. Pharmacologic medical therapies for scleroderma include [[topical]] [[tacrolimus]] for [[morphea]], [[methotrexate]] for diffuse [[sclerosis]] of the [[skin]], [[minocycline]] for [[calcinosis cutis]], [[nifedipine]] for [[Raynaud's phenomenon]], [[captopril]] for scleroderma [[renal]] crisis.

==Historical Perspective==
The word scleroderma comes from greek words; skleros (hard) and derma ([[skin]]). Scleroderma was first described by Carlo Curzio in Naples, Italy in 1753. The association between abnormal [[vasoconstriction]] and diffuse scleroderma was made in 1865 by Raynaud.

==Classification==
Scleroderma ([[systemic sclerosis]]) is classified into 2 subtypes, limited cutaneous scleroderma and diffuse cutaneous scleroderma. [[Morphea]] and [[CREST syndrome]] are variants of limited cutaneous scleroderma. Scleroderma was previously classified according to [[American College of Rheumatology|American College of Rheumatology (ACR)]] 1980 preliminary scleroderma criteria. Scleroderma ([[systemic sclerosis]]) is now classified according to the new [[American College of Rheumatology|American College of Rheumatology (ACR)]]/ European League Against Rheumatism (EULAR) criteria.

==Pathophysiology==
Scleroderma is an [[autoimmune]] [[connective tissue disease]]. The hallmark of the underlying pathophysiology is production of [[autoantibodies]] against various [[cellular]] [[antigens]], small [[vessel]] [[vasculopathy]], [[fibrosis]] of [[skin]] and internal organs, and excess [[collagen]] deposition in the [[skin]] and internal organs. Circulating [[autoantibodies]] found in patients with scleroderma are anti-[[topoisomerase I]] (Scl-70) [[antibody]], [[Anti-centromere antibodies|anti-centromere]] [[antibody]], anti-[[RNA polymerase III]] [[antibody]], anti-nucleolar [[antibody]]. [[Growth factors]] and [[cytokines]] play an important role in the underlying pathogenesis of scleroderma. Increased [[fibroblast]] activity leads to the excessive [[collagen]] deposition in scleroderma. Although the hallmark of this disease is [[skin]] [[fibrosis]], internal organ involvement is a fatal complication and includes, [[esophageal dysmotility]], [[interstitial lung disease]], [[pulmonary arterial hypertension]], scleroderma [[renal]] crisis, [[myocardial]] [[fibrosis]], [[pericardial]] [[fibrosis]] and [[pericardial effusion]]. Although scleroderma occurs in a sporadic pattern in the general population, variations in the [[Human leukocyte antigen|human leukocyte antigen (HLA)]] genes can predispose an individual to developing scleroderma. On gross pathology, [[sclerodactyly]], [[skin]] [[fibrosis]], [[edema]] and [[calcinosis]] are characteristic findings of scleroderma. On [[microscopic]] [[histopathological]] analysis, characteristic findings of scleroderma include microvascular damage, [[Perivascular cell|perivascular]] infiltrates of [[immune]] [[cells]], loss of [[microvasculature]], [[Perivascular cell|perivascular]] [[edema]], [[fibrosis]], densely packed [[collagen]] in the lower [[dermis]] and upper [[subcutaneous]] layer, [[atrophy]] and loss of [[cells]] in the later stages of the disease. The [[endothelial]] cell dysfunction allows the [[chemokine]]- and [[cytokine]]-mediated attraction of [[inflammatory]] cells and [[fibroblast]] precursors ([[fibrocytes]]) from the [[bloodstream]] and [[bone marrow]] and their transmigration into the surrounding tissues, resulting in the establishment of a [[chronic]] [[inflammatory]] process with participation of [[macrophages]] and T and B [[lymphocytes]], with further production and secretion of [[cytokines]] and growth factors from these cells.The [[immunological]] alterations include innate [[immunity]] abnormalities, tissue infiltration with [[macrophages]] and T and B [[lymphocytes]]; production of numerous disease-specific [[autoantibodies]]; and [[dysregulation]] of [[cytokine]], [[chemokine]], and [[growth factor]] production. The released [[cytokines]] and [[growth]] factors induce the activation and [[phenotypic]] conversion of various cellular types, including resident [[fibroblast]]s, [[epithelial]] cells, [[endothelial cell]]s, and [[pericytes]] into activated [[myofibroblast]]s, the cells ultimately responsible for initiation and establishment of the [[fibrotic]] process.

==Causes==
The cause of scleroderma has not been identified. There is a possibility of an underlying [[Immunological|immunologic]] abnormality. To review risk factors for the development of scleroderma [[Scleroderma risk factors|click here.]]

==Differentiating Scleroderma from other Diseases==
Scleroderma must be differentiated from other diseases that cause [[skin]] thickening, [[sclerodactyly]], [[edema]] and symptoms of [[GERD]] such as scleredema, [[scleromyxedema]], [[eosinophilic fasciitis]], chronic [[graft-versus-host disease]], drug induced scleroderma, scleroderma overlap syndromes, [[diabetic]] cheiroarthropathy, [[myxedema]] and [[nephrogenic systemic fibrosis]].

==Epidemiology and Demographics==
The majority of cases of scleroderma have been reported from the United States. The [[prevalence]] of scleroderma is approximately 24 cases per 100,000 individuals in the United States. Scleroderma commonly affects individuals between 20 to 50 years of age. Choctaw native Americans have a much higher [[prevalence]] of scleroderma than the general population. Females are more commonly affected than males. Familial clustering of scleroderma has been reported in United States and Australia.

==Risk Factors==
Common risk factors in the development of scleroderma include occupational and environmental exposure to certain [[chemicals]], certain [[genetic]] variations and [[infectious]] agents. Most commonly implicated occupational and environmental risk factors are exposure is to [[silica]], chlorinated and [[aromatic]] solvents as well as welding [[fumes]].

==Screening==
There is insufficient evidence to recommend routine screening for scleroderma, however screening is recommended for [[pulmonary arterial hypertension]] and [[malignancy]] in scleroderma patients. Regular [[blood pressure]] monitoring at home is encouraged in patients with scleroderma to screen for [[renal]] involvement and prevention of scleroderma [[renal]] crisis

== Natural History, Complications and Prognosis==
If left untreated, patients with scleroderma may progress to develop [[Pulmonary arterial hypertension|pulmonary arterial hypertension (PAH)]], [[interstitial lung disease]] and severe gastrointestinal disease. Common complications of scleroderma include [[pulmonary fibrosis]], [[Pulmonary hypertension|pulmonary arterial hypertension]], [[interstitial lung disease]] and scleroderma renal crisis. The 10-year survival rate of patients with scleroderma is approximately 70%-80%.

== Diagnosis ==

===Diagnostic Study of Choice===
There is no single diagnostic study of choice for the diagnosis of scleroderma. Scleroderma is mainly diagnosed based on clinical presentation, though scleroderma ([[systemic sclerosis]]) maybe diagnosed based on the new [[American College of Rheumatology|American College of Rheumatology (ACR)]]/ European League Against Rheumatism (EULAR) classification criteria. The new criteria is more sensitive and is better for detecting disease earlier, so that the fatal [[renal]] and [[pulmonary]] complications can be screened for and prevented.

===History and Symptoms===
The hallmark of scleroderma is [[sclerodactyly]]. A positive history of progressive [[skin]] tightening and hardening is suggestive of scleroderma. The most common symptoms of scleroderma include [[skin]] tightening or [[induration]], [[Raynaud's phenomenon]], and symptoms of [[gastroesophageal reflux disease]] (GERD). Less common symptoms of scleroderma include shiny [[skin]] appearance and restricted movement of affected areas of the [[skin]].

=== Physical Examination ===
Patients with scleroderma usually appear anxious. Physical examination of patients with scleroderma is usually remarkable for [[sclerodactyly]], [[Raynaud's phenomenon]], digital [[ulcers]], [[skin]] [[fibrosis]] and [[Telangiectasis|telangiectasias]].

===Electrocardiogram===
An ECG may be helpful in the diagnosis of scleroderma [[heart]] disease. Findings on an ECG suggestive of scleroderma heart disease include [[left bundle branch block]], [[right bundle branch block]] and [[septal]] [[infarction]] pattern. It is recommended to screen for [[myocardial]] [[fibrosis]] with an annual electrocardiogram in patients with scleroderma.

===Chest X-Ray===
An [[x-ray]] of the chest may be helpful in the diagnosis of scleroderma [[interstitial lung disease]] and [[pulmonary fibrosis]].Although it is usually not as sensitive as [[HRCT]], findings on an [[x-ray]] suggestive of scleroderma [[interstitial lung disease]] include, [[interstitial]] opacification, reticular areas of [[attenuation]], ground glass opacity greatest at [[lung]] bases.

===CT Scan===
[[High-resolution CT|High-resolution CT (HRCT) scan]] of the chest may be helpful in the diagnosis of scleroderma [[interstitial lung disease]] and [[pulmonary hypertension]]. Findings on [[HRCT]] suggestive of [[interstitial lung disease]] include architectural distortion due to [[pulmonary fibrosis]], reticular interlobular [[interstitial]] thickening, increased ground glass opacity and accentuated reticular markings on juxtapleural, [[posterior]] and basilar portion of the [[lungs]], traction [[bronchiectasis]], and honeycomb cystic change

===MRI===
There are no MRI findings associated with scleroderma.

===Echocardiography or Ultrasound===
There are no [[echocardiography]]/[[ultrasound]] findings associated with scleroderma. However, a [[Transthoracic echocardiography|transthoracic echocardiography (TTE)]] may be helpful in the diagnosis of complications of scleroderma, which include [[Pulmonary arterial hypertension|pulmonary arterial hypertension (PAH)]].

===Other Imaging Findings===
Nail-fold video capillaroscopy (NVC) may be helpful in the diagnosis of scleroderma. Findings on nail-fold video capillaroscopy diagnostic of [[Raynaud's phenomenon|raynaud's phenomenon (RP)]] and scleroderma [[microangiopathy]] include nail-fold capillary abnormalities, [[capillary]] dilatation, and [[capillary]] loop drop-out. Findings on nail-fold video capillaroscopy diagnostic of scleroderma [[microangiopathy]] are graded into 3 phases; early, active and late.

===Other Diagnostic Studies===
There are no other diagnostic study associated with scleroderma.

==Treatment==

===Medical Therapy===
The mainstay of treatment for scleroderma is medical therapy. Pharmacologic medical therapies for scleroderma include [[topical]] [[tacrolimus]] for [[morphea]], [[methotrexate]] for diffuse [[sclerosis]] of the [[skin]], [[minocycline]] for [[calcinosis cutis]], [[nifedipine]] for [[Raynaud's phenomenon]], [[captopril]] for scleroderma [[renal]] crisis, treatment of [[gastroesophageal reflux disease]] and [[pulmonary hypertension]]. Localized [[phototherapy]] with [[ultraviolet light]] is preferred for the treatment of [[morphea]].

===Surgery===
Surgical [[Intervention (counseling)|intervention]] is not recommended for the management of scleroderma. However, surgical procedures such as [[debulking]] and [[lung transplantation]] are needed to treat complications of scleroderma which include [[calcinosis cutis]] and [[pulmonary hypertension]].

===Primary Prevention===
Effective measures for the primary prevention of scleroderma include avoiding occupational and environmental exposure to crystalline [[silica]], [[epoxy resins]], welding [[fumes]] and hand-arm [[vibration]].

===Secondary Prevention===
There are no established measures for the secondary prevention of scleroderma. However, effective measures for the secondary prevention of [[pulmonary arterial hypertension]] and scleroderma [[renal]] crisis in patients with scleroderma include [[Screening test|screening]].

==References==
{{Reflist|2}}

[[Category:Up-To-Date]]
[[Category:Primary care]]
[[Category:Medicine]]
[[Category:Dermatology]]
[[Category:Rheumatology]]

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

Scleroderma overview

2020-05-10T22:02:47Z

Ayesha A. Khan: /* Pathophysiology */

__NOTOC__
{{Scleroderma}}
{{CMG}}; {{AE}} {{MKA}}

==Overview==
Scleroderma is an [[autoimmune]] [[connective tissue disease]]. The hallmark of the disease is the presence of [[autoantibodies]] against various [[cellular]] [[antigens]], which results in small [[vessel]] [[vasculopathy]], excessive [[collagen]] deposition and [[fibrosis]] of [[skin]] and internal organs. [[Growth factors]] and [[cytokines]] play an important role in the underlying [[pathogenesis]]. The word scleroderma is greek in origin and translates in to hard [[skin]], describing the [[skin]] [[fibrosis]]. Scleroderma is classified into 2 subtype, limited cutaneous scleroderma and diffuse [[cutaneous]] scleroderma. [[CREST syndrome]] is a variant of limited [[cutaneous]] scleroderma. Choctaw native Americans have a much higher [[prevalence]] of scleroderma than the general population. Scleroderma must be differentiated from other diseases that cause [[skin]] thickening, [[sclerodactyly]], [[edema]] and symptoms of [[gastroesophageal reflux disease]]. Common risk factors for scleroderma that have been described in literature are exposure to [[silica]], chlorinated and [[aromatic]] solvents and welding [[fumes]]. Since there is a possibility of internal organ involvement, screening for fatal complications such as scleroderma [[renal]] crisis, [[pulmonary arterial hypertension]], and [[cardiovascular]] involvement must be carried out in patients with scleroderma. Scleroderma is mainly diagnosed based on clinical presentation and the new [[American College of Rheumatology|American College of Rheumatology (ACR)]]/ European League Against Rheumatism (EULAR) classification criteria has been published. The new criteria is more sensitive and is better for detecting disease earlier, so that the fatal [[renal]] and [[pulmonary]] complications can be screened for and prevented. Nailfold video capillaroscopy is the diagnostic study of choice to confirm [[Raynaud's phenomenon]] and scleroderma [[microangiopathy]]. The mainstay of treatment for scleroderma is medical therapy. Pharmacologic medical therapies for scleroderma include [[topical]] [[tacrolimus]] for [[morphea]], [[methotrexate]] for diffuse [[sclerosis]] of the [[skin]], [[minocycline]] for [[calcinosis cutis]], [[nifedipine]] for [[Raynaud's phenomenon]], [[captopril]] for scleroderma [[renal]] crisis.

==Historical Perspective==
The word scleroderma comes from greek words; skleros (hard) and derma ([[skin]]). Scleroderma was first described by Carlo Curzio in Naples, Italy in 1753. The association between abnormal [[vasoconstriction]] and diffuse scleroderma was made in 1865 by Raynaud.

==Classification==
Scleroderma ([[systemic sclerosis]]) is classified into 2 subtypes, limited cutaneous scleroderma and diffuse cutaneous scleroderma. [[Morphea]] and [[CREST syndrome]] are variants of limited cutaneous scleroderma. Scleroderma was previously classified according to [[American College of Rheumatology|American College of Rheumatology (ACR)]] 1980 preliminary scleroderma criteria. Scleroderma ([[systemic sclerosis]]) is now classified according to the new [[American College of Rheumatology|American College of Rheumatology (ACR)]]/ European League Against Rheumatism (EULAR) criteria.

==Pathophysiology==
Scleroderma is an [[autoimmune]] [[connective tissue disease]]. The hallmark of the underlying pathophysiology is production of [[autoantibodies]] against various [[cellular]] [[antigens]], small [[vessel]] [[vasculopathy]], [[fibrosis]] of [[skin]] and internal organs, and excess [[collagen]] deposition in the [[skin]] and internal organs. Circulating [[autoantibodies]] found in patients with scleroderma are anti-[[topoisomerase I]] (Scl-70) [[antibody]], [[Anti-centromere antibodies|anti-centromere]] [[antibody]], anti-[[RNA polymerase III]] [[antibody]], anti-nucleolar [[antibody]]. [[Growth factors]] and [[cytokines]] play an important role in the underlying pathogenesis of scleroderma. Increased [[fibroblast]] activity leads to the excessive [[collagen]] deposition in scleroderma. Although the hallmark of this disease is [[skin]] [[fibrosis]], internal organ involvement is a fatal complication and includes, [[esophageal dysmotility]], [[interstitial lung disease]], [[pulmonary arterial hypertension]], scleroderma [[renal]] crisis, [[myocardial]] [[fibrosis]], [[pericardial]] [[fibrosis]] and [[pericardial effusion]]. Although scleroderma occurs in a sporadic pattern in the general population, variations in the [[Human leukocyte antigen|human leukocyte antigen (HLA)]] genes can predispose an individual to developing scleroderma. On gross pathology, [[sclerodactyly]], [[skin]] [[fibrosis]], [[edema]] and [[calcinosis]] are characteristic findings of scleroderma. On [[microscopic]] [[histopathological]] analysis, characteristic findings of scleroderma include microvascular damage, [[Perivascular cell|perivascular]] infiltrates of [[immune]] [[cells]], loss of [[microvasculature]], [[Perivascular cell|perivascular]] [[edema]], [[fibrosis]], densely packed [[collagen]] in the lower [[dermis]] and upper [[subcutaneous]] layer, [[atrophy]] and loss of [[cells]] in the later stages of the disease. The [[endothelial]] cell dysfunction allows the [[chemokine]]- and [[cytokine]]-mediated attraction of [[inflammatory]] cells and [[fibroblast]] precursors ([[fibrocytes]]) from the [[bloodstream]] and [[bone marrow]] and their transmigration into the surrounding tissues, resulting in the establishment of a [[chronic]] [[inflammatory]] process with participation of [[macrophages]] and T and B [[lymphocytes]], with further production and secretion of [[cytokines]] and growth factors from these cells.The [[immunological]] alterations include innate [[immunity]] abnormalities, tissue infiltration with [[macrophages]] and T and B [[lymphocytes]]; production of numerous disease-specific [[autoantibodies]]; and [[dysregulation]] of [[cytokine]], [[chemokine]], and [[growth factor]] production. The released [[cytokines]] and [[growth]] factors induce the activation and [[phenotypic]] conversion of various cellular types, including resident [[fibroblast]]s, [[epithelial]] cells, [[endothelial cell]]s, and [[pericytes]] into activated [[myofibroblast]]s, the cells ultimately responsible for initiation and establishment of the [[fibrotic]] process.

==Causes==
The cause of scleroderma has not been identified. There is a possibility of an underlying [[Immunological|immunologic]] abnormality. To review risk factors for the development of scleroderma [[Scleroderma risk factors|click here.]]

==Differentiating Scleroderma from other Diseases==
Scleroderma must be differentiated from other diseases that cause [[skin]] thickening, [[sclerodactyly]], [[edema]] and symptoms of [[GERD]] such as scleredema, [[scleromyxedema]], [[eosinophilic fasciitis]], chronic [[graft-versus-host disease]], drug induced scleroderma, scleroderma overlap syndromes, [[diabetic]] cheiroarthropathy, [[myxedema]] and [[nephrogenic systemic fibrosis]].

==Epidemiology and Demographics==
The majority of cases of scleroderma have been reported from the United States. The [[prevalence]] of scleroderma is approximately 24 cases per 100,000 individuals in the United States. Scleroderma commonly affects individuals between 20 to 50 years of age. Choctaw native Americans have a much higher [[prevalence]] of scleroderma than the general population. Females are more commonly affected than males. Familial clustering of scleroderma has been reported in United States and Australia.

==Risk Factors==
Common risk factors in the development of scleroderma include occupational and environmental exposure to certain [[chemicals]], certain [[genetic]] variations and [[infectious]] agents. Most commonly implicated occupational and environmental risk factors are exposure is to [[silica]], chlorinated and [[aromatic]] solvents as well as welding [[fumes]].

==Screening==
There is insufficient evidence to recommend routine screening for scleroderma, however screening is recommended for [[pulmonary arterial hypertension]] and [[malignancy]] in scleroderma patients. Regular [[blood pressure]] monitoring at home is encouraged in patients with scleroderma to screen for [[renal]] involvement and prevention of scleroderma [[renal]] crisis

== Natural History, Complications and Prognosis==
If left untreated, patients with scleroderma may progress to develop [[Pulmonary arterial hypertension|pulmonary arterial hypertension (PAH)]], [[interstitial lung disease]] and severe gastrointestinal disease. Common complications of scleroderma include [[pulmonary fibrosis]], [[Pulmonary hypertension|pulmonary arterial hypertension]], [[interstitial lung disease]] and scleroderma renal crisis. The 10-year survival rate of patients with scleroderma is approximately 70%-80%.

== Diagnosis ==

===Diagnostic Study of Choice===
There is no single diagnostic study of choice for the diagnosis of scleroderma. Scleroderma is mainly diagnosed based on clinical presentation, though scleroderma ([[systemic sclerosis]]) maybe diagnosed based on the new [[American College of Rheumatology|American College of Rheumatology (ACR)]]/ European League Against Rheumatism (EULAR) classification criteria. The new criteria is more sensitive and is better for detecting disease earlier, so that the fatal [[renal]] and [[pulmonary]] complications can be screened for and prevented.

===History and Symptoms===
The hallmark of scleroderma is [[sclerodactyly]]. A positive history of progressive [[skin]] tightening and hardening is suggestive of scleroderma. The most common symptoms of scleroderma include [[skin]] tightening or [[induration]], [[Raynaud's phenomenon]], and symptoms of [[gastroesophageal reflux disease]] (GERD). Less common symptoms of scleroderma include shiny [[skin]] appearance and restricted movement of affected areas of the [[skin]].

=== Physical Examination ===
Patients with scleroderma usually appear anxious. Physical examination of patients with scleroderma is usually remarkable for [[sclerodactyly]], [[Raynaud's phenomenon]], digital [[ulcers]], [[skin]] [[fibrosis]] and [[Telangiectasis|telangiectasias]].

===Electrocardiogram===
An ECG may be helpful in the diagnosis of scleroderma [[heart]] disease. Findings on an ECG suggestive of scleroderma heart disease include [[left bundle branch block]], [[right bundle branch block]] and [[septal]] [[infarction]] pattern. It is recommended to screen for [[myocardial]] [[fibrosis]] with an annual electrocardiogram in patients with scleroderma.

===Chest X-Ray===
An [[x-ray]] of the chest may be helpful in the diagnosis of scleroderma [[interstitial lung disease]] and [[pulmonary fibrosis]].Although it is usually not as sensitive as [[HRCT]], findings on an [[x-ray]] suggestive of scleroderma [[interstitial lung disease]] include, [[interstitial]] opacification, reticular areas of [[attenuation]], ground glass opacity greatest at [[lung]] bases.

===CT Scan===
[[High-resolution CT|High-resolution CT (HRCT) scan]] of the chest may be helpful in the diagnosis of scleroderma [[interstitial lung disease]] and [[pulmonary hypertension]]. Findings on [[HRCT]] suggestive of [[interstitial lung disease]] include architectural distortion due to [[pulmonary fibrosis]], reticular interlobular [[interstitial]] thickening, increased ground glass opacity and accentuated reticular markings on juxtapleural, [[posterior]] and basilar portion of the [[lungs]], traction [[bronchiectasis]], and honeycomb cystic change

===MRI===
There are no MRI findings associated with scleroderma.

===Echocardiography or Ultrasound===
There are no [[echocardiography]]/[[ultrasound]] findings associated with scleroderma. However, a [[Transthoracic echocardiography|transthoracic echocardiography (TTE)]] may be helpful in the diagnosis of complications of scleroderma, which include [[Pulmonary arterial hypertension|pulmonary arterial hypertension (PAH)]].

===Other Imaging Findings===
Nail-fold video capillaroscopy (NVC) may be helpful in the diagnosis of scleroderma. Findings on nail-fold video capillaroscopy diagnostic of [[Raynaud's phenomenon|raynaud's phenomenon (RP)]] and scleroderma [[microangiopathy]] include nail-fold capillary abnormalities, [[capillary]] dilatation, and [[capillary]] loop drop-out. Findings on nail-fold video capillaroscopy diagnostic of scleroderma [[microangiopathy]] are graded into 3 phases; early, active and late.

===Other Diagnostic Studies===
There are no other diagnostic study associated with scleroderma.

==Treatment==

===Medical Therapy===
The mainstay of treatment for scleroderma is medical therapy. Pharmacologic medical therapies for scleroderma include [[topical]] [[tacrolimus]] for [[morphea]], [[methotrexate]] for diffuse [[sclerosis]] of the [[skin]], [[minocycline]] for [[calcinosis cutis]], [[nifedipine]] for [[Raynaud's phenomenon]], [[captopril]] for scleroderma [[renal]] crisis, treatment of [[gastroesophageal reflux disease]] and [[pulmonary hypertension]]. Localized [[phototherapy]] with [[ultraviolet light]] is preferred for the treatment of [[morphea]].

===Surgery===
Surgical [[Intervention (counseling)|intervention]] is not recommended for the management of scleroderma. However, surgical procedures such as [[debulking]] and [[lung transplantation]] are needed to treat complications of scleroderma which include [[calcinosis cutis]] and [[pulmonary hypertension]].

===Primary Prevention===
Effective measures for the primary prevention of scleroderma include avoiding occupational and environmental exposure to crystalline [[silica]], [[epoxy resins]], welding [[fumes]] and hand-arm [[vibration]].

===Secondary Prevention===
There are no established measures for the secondary prevention of scleroderma. However, effective measures for the secondary prevention of [[pulmonary arterial hypertension]] and scleroderma [[renal]] crisis in patients with scleroderma include [[Screening test|screening]].

==References==
{{Reflist|2}}

[[Category:Up-To-Date]]
[[Category:Primary care]]
[[Category:Medicine]]
[[Category:Dermatology]]
[[Category:Rheumatology]]

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

Sjögren's syndrome overview

2020-05-10T18:23:11Z

Ayesha A. Khan:

__NOTOC__
{{Sjögren's syndrome}}
{{CMG}} {{AE}} {{F.K}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com]

==Overview==
In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with enlargement of the [[parotid]] and [[lacrimal glands]] associated with a round-cell infiltrate and [[acinar]] [[atrophy]]. In 1933, Henrik Sjögren was the first to describe 19 females with clinical and pathological manifestations of the Sjögren's syndrome. American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to [[clinical]] findings. Common causes of Sjögren's syndrome include [[viral infection]] such as [[Epstein Barr virus|Epstein-Barr virus]] (EBV), [[Coxsackie virus]], [[Hepatitis C virus]], [[Cytomegalovirus]] (CMV), [[Human herpesvirus 6]] (HHV-6), [[Retroviruses]], and [[genetic]] factors. The [[incidence]] of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The [[prevalence]] of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California. Common risk factors in the development of Sjögren's syndrome include family history of [[autoimmune diseases]], serological markers such as low [[complement]] levels and [[cryoglobulinemia]] and [[parotid gland enlargement]]. The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as [[ocular]] and [[oral]] dryness. Common complications of Sjögren's syndrome include [[blurred vision]] and [[corneal]] damage, [[optic neuritis]], and [[lymphoma]]. [[Prognosis]] is generally good and presence of low [[complement]] level is associated with a particularly poor [[prognosis]] among patients with Sjögren's syndrome. The most common symptoms of Sjögren's syndrome include [[ocular]] and [[oral]] symptoms. Patients with Sjögren's syndrome may have a positive history of [[rheumatoid arthritis]] (RA), [[systemic lupus erythematous]] (SLE) and [[non-Hodgkin B-cell lymphoma]]. Physical examination of patients with is usually remarkable for dryness of all [[mucous membranes]] such as [[mouth]], [[eyes]], [[lips]], [[anal]] and [[rectal]]. Laboratory findings consistent with the diagnosis of Sjögren's syndrome include elevated [[erythrocyte sedimentation rate]] ([[Erythrocyte sedimentation rate|ESR]]), [[cytopenia]], the presence of anti-SSA/Ro, anti-SSB/La. Findings on an [[ultrasound]] suggestive of Sjögren's syndrome are hypoechoic and inhomogeneous [[salivary glands]], [[parenchymal]] inhomogeneity in the [[submandibular]] glands and focal or diffuse hypoechoic or anechoic foci in [[Gland|glands]]. [[Parotid gland]] [[Computed tomography|CT scan]] may be helpful in the diagnosis of Sjögren's syndrome and finding include abnormal [[diffuse]] [[Adipose tissue|fat tissue]] deposition and diffuse punctate [[calcification]]. The most commonly used tests for [[dry eyes]] of Sjögren's syndrome include [[Schirmer's test]], [[ocular]] surface staining and tear break-up time. Pharmacologic medical therapies for [[Keratoconjunctivitis sicca|dry eye]], [[Xerostomia|dry mouth]], and other sicca symptoms of Sjögren's syndrome are [[pilocarpine]], [[cevimeline]] and [[artificial tears]]. The mainstay of treatment for Sjögren's syndrome is medical therapy. Surgery is usually reserved for patients with [[occlusion]] of the lacrimal puncta, [[salivary gland]] [[malignancy]] and recurrent [[parotitis]] refractory to medical management.

==Historical Perspective==
In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with with enlargement of the [[parotid]] and [[lacrimal glands]] associated with a round-cell infiltrate and [[acinar]] [[atrophy]]. In 1933, Henrik Sjögren was the first to describe 19 females with clinical and [[Pathology (disambiguation)|pathological]] manifestations of the Sjögren's syndrome.

==Classification==
American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to [[clinical]] findings.

==Pathophysiology==
[[Sjögren's syndrome]] (SS) is a chronic [[Autoimmunity|autoimmune disorder]] that can affect several [[Organ (anatomy)|organ systems]]. [[Sjögren's syndrome]] is classified into a "primary" form that is a separate entity from other well-defined [[Autoimmune disease|autoimmune]] disorders and a "secondary" form that is associated with other well-defined [[Autoimmunity|autoimmune]] conditions, such as [[SLE]], [[rheumatoid arthritis]], [[Progressive Systemic Sclerosis|progressive systemic sclerosis]], and [[primary biliary cirrhosis]]. These forms of [[Sjögren's syndrome]] are different in their [[Serology|serologic]] and [[Histopathology|histopathologic]] findings as well as their [[Genetics|genetic]] components. Both [[Genetics|genetic]] and immune factors contribute to the [[pathogenesis]] of the disease. In the most common presentation of [[Sjögren's syndrome]], [[Lymphocyte|lymphocytes]] infiltrate the [[lacrimal]] and [[Salivary gland|salivary glands]] and impair their function, hence causing the main characteristic [[Symptom|symptoms]] such as [[Xerostomia|dry mouth]] ([[xerostomia]]) and [[dry eyes]] ([[keratoconjunctivitis sicca]]). [[CD4|CD4+]] [[T cell|T-cells]] are predominant in mild and moderate [[salivary gland]] infiltrations, while [[B cell|B cells]] play the major role in severe lesions. [[Sjögren's syndrome]] may also manifest itself with dryness of [[skin]] and other [[Mucous membrane|mucosal surfaces]] or even cause systemic extraglandular disturbances such as [[arthritis]], [[vasculitis]], [[Kidney|renal]], [[Lung|pulmonary]], [[Hematopoiesis|hematopoietic]], and [[Neurology|neurologic]] involvement. In general, a combination of [[Lymphocyte|lymphocytic]] infiltration, [[B cell|B lymphocyte]] hyperreactivity, production of certain [[Autoantibody|autoantibodies]], [[Gene|genes]] mostly involved in the production of [[MHC]] molecules and certain [[Virus|viral infections]] which <nowiki/>are all linked to the [[pathogenesis]] of [[Sjögren's syndrome]].

==Causes==
Common causes of Sjögren's syndrome include [[Infection|viral infection]] such as [[Epstein Barr virus|Epstein-Barr virus]] (EBV), [[Coxsackie virus]], [[Hepatitis C virus]], [[Cytomegalovirus]] (CMV), [[Human herpesvirus 6]] (HHV-6), [[Retroviruses]] and [[genetic]] factors.

==Differentiating {{PAGENAME}} from Other Diseases==

==Epidemiology and Demographics==
The [[incidence]] of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The [[prevalence]] of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. The [[mortality rate]] of Sjögren's syndrome is approximately 1.38. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California.

==Risk Factors==
Common risk factors in the development of Sjögren's syndrome include family history of [[autoimmune diseases]], serological markers such as low [[complement]] levels and [[cryoglobulinemia]] and [[parotid gland enlargement]].

==Screening==
There is insufficient evidence to recommend routine screening for Sjögren's syndrome.

==Natural History, Complications, and Prognosis==
===Natural History===
The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as [[ocular]] and [[oral]] dryness.
===Complications===
Common complications of Sjögren's syndrome include [[blurred vision]] and [[corneal]] damage, [[optic neuritis]] and [[lymphoma]].
===Prognosis===
[[Prognosis]] is generally good and presence of low [[complement]] level is associated with a particularly poor [[prognosis]] among patients with Sjögren's syndrome.

==Diagnosis==
===Diagnostic Study of Choice===

===History and Symptoms===
The most common symptoms of Sjögren's syndrome include [[ocular]] and [[Mouth|oral]] symptoms. Patients with Sjögren's syndrome may have a positive history of [[rheumatoid arthritis]] ([[Rheumatoid arthritis|RA]]), [[systemic lupus erythematous]] ([[SLE]]) and [[Non-Hodgkin lymphoma|non-Hodgkin B-cell lymphoma]].

===Physical Examination===
Physical examination of patients with is usually remarkable for dryness of all [[mucous membranes]] such as [[mouth]], [[Eye|eyes]], lips, [[anal]], and [[rectal]].

===Laboratory Findings===
Laboratory findings consistent with the diagnosis of Sjögren's syndrome include elevated [[erythrocyte sedimentation rate]] ([[Erythrocyte sedimentation rate|ESR]]), [[cytopenia]], presence of anti-SSA/Ro, and anti-SSB/La.

===Electrocardiogram===
There are no ECG findings associated with Sjögren's syndrome.

=== X-ray ===

=== Echocardiography and Ultrasound ===
Findings on an [[ultrasound]] suggestive of Sjögren's syndrome are hypoechoic and inhomogeneous [[salivary glands]], [[parenchymal]] inhomogeneity in the [[submandibular]] glands and focal or diffuse hypoechoic or anechoic foci in glands. There are no [[echocardiography]] findings associated with Sjögren's syndrome.

=== CT scan ===
[[Parotid gland]] [[Computed tomography|CT scan]] may be helpful in the diagnosis of Sjögren's syndrome and finding include abnormal diffuse [[Adipose tissue|fat tissue]] deposition and diffuse punctate [[calcification]].

=== MRI ===
Findings on [[Magnetic resonance imaging|MRI]] suggestive of Sjögren's syndrome include loss of homogeneity kin signal intensity on T1-weighted [[MR]] images and fat saturation suppressed focal high-intensity areas on T1-weighted images.

=== Other Imaging Findings ===
There are no other imaging findings associated with Sjögren's syndrome.

=== Other Diagnostic Studies ===
The most commonly used tests for dry eyes of Sjögren's syndrome include [[Schirmer's test]], [[ocular]] surface staining and [[Tears|tear]] break-up time.

==Treatment==
===Medical Therapy===
Pharmacologic medical therapies for [[Keratoconjunctivitis sicca|dry eye]], [[Xerostomia|dry mouth]], and other sicca symptom of Sjögren's syndrome are [[pilocarpine]], [[cevimeline]] and [[artificial tears]].

===Surgery===
The mainstay of treatment for Sjögren's syndrome is medical therapy. Surgery is usually reserved for patients with [[occlusion]] of the lacrimal puncta, [[salivary gland]] [[malignancy]], and recurrent [[parotitis]] refractory to medical management.

===Primary Prevention===
[[Sjögren's syndrome]] is [[inherited]] condition, there is no particular way to prevent developing the disease.

=== Secondary Prevention ===
[[Secondary prevention]] by [[Smoking]] cessation. Smoking can irritate and [[dry]] out your mouth. Increase your [[fluid]] intake. Take sips of fluids, particularly water, throughout the day. Avoid drinking coffee or [[alcohol]] since they can worsen dry mouth symptoms. Also avoid acidic beverages such as colas and some sports drinks because the acid can harm the enamel of your teeth. Stimulate [[saliva]] flow. Sugarless gum or citrus-flavored hard candies can boost [[saliva]] flow. Because [[Sjogren's syndrome]] increases your risk of [[dental]] cavities, limit sweets, especially between meals. Try artificial saliva. [[Saliva]] replacement products often work better than plain water because they contain a lubricant that helps your [[mouth]] stay moist longer. These products come as a spray or lozenge.

==References==
{{reflist|2}}

[[Category:Medicine]]
[[Category:Immunology]]
[[Category:Rheumatology]]
[[Category:Up-To-Date]]
[[Category:Primary care]]

Sjögren's syndrome overview

2020-05-10T18:22:09Z

Ayesha A. Khan: /* Secondary Prevention */

__NOTOC__
{{Sjögren's syndrome}}
{{CMG}} {{AE}} {{F.K}}

==Overview==
In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with enlargement of the [[parotid]] and [[lacrimal glands]] associated with a round-cell infiltrate and [[acinar]] [[atrophy]]. In 1933, Henrik Sjögren was the first to describe 19 females with clinical and pathological manifestations of the Sjögren's syndrome. American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to [[clinical]] findings. Common causes of Sjögren's syndrome include [[viral infection]] such as [[Epstein Barr virus|Epstein-Barr virus]] (EBV), [[Coxsackie virus]], [[Hepatitis C virus]], [[Cytomegalovirus]] (CMV), [[Human herpesvirus 6]] (HHV-6), [[Retroviruses]], and [[genetic]] factors. The [[incidence]] of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The [[prevalence]] of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California. Common risk factors in the development of Sjögren's syndrome include family history of [[autoimmune diseases]], serological markers such as low [[complement]] levels and [[cryoglobulinemia]] and [[parotid gland enlargement]]. The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as [[ocular]] and [[oral]] dryness. Common complications of Sjögren's syndrome include [[blurred vision]] and [[corneal]] damage, [[optic neuritis]], and [[lymphoma]]. [[Prognosis]] is generally good and presence of low [[complement]] level is associated with a particularly poor [[prognosis]] among patients with Sjögren's syndrome. The most common symptoms of Sjögren's syndrome include [[ocular]] and [[oral]] symptoms. Patients with Sjögren's syndrome may have a positive history of [[rheumatoid arthritis]] (RA), [[systemic lupus erythematous]] (SLE) and [[non-Hodgkin B-cell lymphoma]]. Physical examination of patients with is usually remarkable for dryness of all [[mucous membranes]] such as [[mouth]], [[eyes]], [[lips]], [[anal]] and [[rectal]]. Laboratory findings consistent with the diagnosis of Sjögren's syndrome include elevated [[erythrocyte sedimentation rate]] ([[Erythrocyte sedimentation rate|ESR]]), [[cytopenia]], the presence of anti-SSA/Ro, anti-SSB/La. Findings on an [[ultrasound]] suggestive of Sjögren's syndrome are hypoechoic and inhomogeneous [[salivary glands]], [[parenchymal]] inhomogeneity in the [[submandibular]] glands and focal or diffuse hypoechoic or anechoic foci in [[Gland|glands]]. [[Parotid gland]] [[Computed tomography|CT scan]] may be helpful in the diagnosis of Sjögren's syndrome and finding include abnormal [[diffuse]] [[Adipose tissue|fat tissue]] deposition and diffuse punctate [[calcification]]. The most commonly used tests for [[dry eyes]] of Sjögren's syndrome include [[Schirmer's test]], [[ocular]] surface staining and tear break-up time. Pharmacologic medical therapies for [[Keratoconjunctivitis sicca|dry eye]], [[Xerostomia|dry mouth]], and other sicca symptoms of Sjögren's syndrome are [[pilocarpine]], [[cevimeline]] and [[artificial tears]]. The mainstay of treatment for Sjögren's syndrome is medical therapy. Surgery is usually reserved for patients with [[occlusion]] of the lacrimal puncta, [[salivary gland]] [[malignancy]] and recurrent [[parotitis]] refractory to medical management.

==Historical Perspective==
In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with with enlargement of the [[parotid]] and [[lacrimal glands]] associated with a round-cell infiltrate and [[acinar]] [[atrophy]]. In 1933, Henrik Sjögren was the first to describe 19 females with clinical and [[Pathology (disambiguation)|pathological]] manifestations of the Sjögren's syndrome.

==Classification==
American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to [[clinical]] findings.

==Pathophysiology==
[[Sjögren's syndrome]] (SS) is a chronic [[Autoimmunity|autoimmune disorder]] that can affect several [[Organ (anatomy)|organ systems]]. [[Sjögren's syndrome]] is classified into a "primary" form that is a separate entity from other well-defined [[Autoimmune disease|autoimmune]] disorders and a "secondary" form that is associated with other well-defined [[Autoimmunity|autoimmune]] conditions, such as [[SLE]], [[rheumatoid arthritis]], [[Progressive Systemic Sclerosis|progressive systemic sclerosis]], and [[primary biliary cirrhosis]]. These forms of [[Sjögren's syndrome]] are different in their [[Serology|serologic]] and [[Histopathology|histopathologic]] findings as well as their [[Genetics|genetic]] components. Both [[Genetics|genetic]] and immune factors contribute to the [[pathogenesis]] of the disease. In the most common presentation of [[Sjögren's syndrome]], [[Lymphocyte|lymphocytes]] infiltrate the [[lacrimal]] and [[Salivary gland|salivary glands]] and impair their function, hence causing the main characteristic [[Symptom|symptoms]] such as [[Xerostomia|dry mouth]] ([[xerostomia]]) and [[dry eyes]] ([[keratoconjunctivitis sicca]]). [[CD4|CD4+]] [[T cell|T-cells]] are predominant in mild and moderate [[salivary gland]] infiltrations, while [[B cell|B cells]] play the major role in severe lesions. [[Sjögren's syndrome]] may also manifest itself with dryness of [[skin]] and other [[Mucous membrane|mucosal surfaces]] or even cause systemic extraglandular disturbances such as [[arthritis]], [[vasculitis]], [[Kidney|renal]], [[Lung|pulmonary]], [[Hematopoiesis|hematopoietic]], and [[Neurology|neurologic]] involvement. In general, a combination of [[Lymphocyte|lymphocytic]] infiltration, [[B cell|B lymphocyte]] hyperreactivity, production of certain [[Autoantibody|autoantibodies]], [[Gene|genes]] mostly involved in the production of [[MHC]] molecules and certain [[Virus|viral infections]] which <nowiki/>are all linked to the [[pathogenesis]] of [[Sjögren's syndrome]].

==Causes==
Common causes of Sjögren's syndrome include [[Infection|viral infection]] such as [[Epstein Barr virus|Epstein-Barr virus]] (EBV), [[Coxsackie virus]], [[Hepatitis C virus]], [[Cytomegalovirus]] (CMV), [[Human herpesvirus 6]] (HHV-6), [[Retroviruses]] and [[genetic]] factors.

==Differentiating {{PAGENAME}} from Other Diseases==

==Epidemiology and Demographics==
The [[incidence]] of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The [[prevalence]] of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. The [[mortality rate]] of Sjögren's syndrome is approximately 1.38. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California.

==Risk Factors==
Common risk factors in the development of Sjögren's syndrome include family history of [[autoimmune diseases]], serological markers such as low [[complement]] levels and [[cryoglobulinemia]] and [[parotid gland enlargement]].

==Screening==
There is insufficient evidence to recommend routine screening for Sjögren's syndrome.

==Natural History, Complications, and Prognosis==
===Natural History===
The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as [[ocular]] and [[oral]] dryness.
===Complications===
Common complications of Sjögren's syndrome include [[blurred vision]] and [[corneal]] damage, [[optic neuritis]] and [[lymphoma]].
===Prognosis===
[[Prognosis]] is generally good and presence of low [[complement]] level is associated with a particularly poor [[prognosis]] among patients with Sjögren's syndrome.

==Diagnosis==
===Diagnostic Study of Choice===

===History and Symptoms===
The most common symptoms of Sjögren's syndrome include [[ocular]] and [[Mouth|oral]] symptoms. Patients with Sjögren's syndrome may have a positive history of [[rheumatoid arthritis]] ([[Rheumatoid arthritis|RA]]), [[systemic lupus erythematous]] ([[SLE]]) and [[Non-Hodgkin lymphoma|non-Hodgkin B-cell lymphoma]].

===Physical Examination===
Physical examination of patients with is usually remarkable for dryness of all [[mucous membranes]] such as [[mouth]], [[Eye|eyes]], lips, [[anal]], and [[rectal]].

===Laboratory Findings===
Laboratory findings consistent with the diagnosis of Sjögren's syndrome include elevated [[erythrocyte sedimentation rate]] ([[Erythrocyte sedimentation rate|ESR]]), [[cytopenia]], presence of anti-SSA/Ro, and anti-SSB/La.

===Electrocardiogram===
There are no ECG findings associated with Sjögren's syndrome.

=== X-ray ===

=== Echocardiography and Ultrasound ===
Findings on an [[ultrasound]] suggestive of Sjögren's syndrome are hypoechoic and inhomogeneous [[salivary glands]], [[parenchymal]] inhomogeneity in the [[submandibular]] glands and focal or diffuse hypoechoic or anechoic foci in glands. There are no [[echocardiography]] findings associated with Sjögren's syndrome.

=== CT scan ===
[[Parotid gland]] [[Computed tomography|CT scan]] may be helpful in the diagnosis of Sjögren's syndrome and finding include abnormal diffuse [[Adipose tissue|fat tissue]] deposition and diffuse punctate [[calcification]].

=== MRI ===
Findings on [[Magnetic resonance imaging|MRI]] suggestive of Sjögren's syndrome include loss of homogeneity kin signal intensity on T1-weighted [[MR]] images and fat saturation suppressed focal high-intensity areas on T1-weighted images.

=== Other Imaging Findings ===
There are no other imaging findings associated with Sjögren's syndrome.

=== Other Diagnostic Studies ===
The most commonly used tests for dry eyes of Sjögren's syndrome include [[Schirmer's test]], [[ocular]] surface staining and [[Tears|tear]] break-up time.

==Treatment==
===Medical Therapy===
Pharmacologic medical therapies for [[Keratoconjunctivitis sicca|dry eye]], [[Xerostomia|dry mouth]], and other sicca symptom of Sjögren's syndrome are [[pilocarpine]], [[cevimeline]] and [[artificial tears]].

===Surgery===
The mainstay of treatment for Sjögren's syndrome is medical therapy. Surgery is usually reserved for patients with [[occlusion]] of the lacrimal puncta, [[salivary gland]] [[malignancy]], and recurrent [[parotitis]] refractory to medical management.

===Primary Prevention===
[[Sjögren's syndrome]] is [[inherited]] condition, there is no particular way to prevent developing the disease.

=== Secondary Prevention ===
[[Secondary prevention]] by [[Smoking]] cessation. Smoking can irritate and [[dry]] out your mouth. Increase your [[fluid]] intake. Take sips of fluids, particularly water, throughout the day. Avoid drinking coffee or [[alcohol]] since they can worsen dry mouth symptoms. Also avoid acidic beverages such as colas and some sports drinks because the acid can harm the enamel of your teeth. Stimulate [[saliva]] flow. Sugarless gum or citrus-flavored hard candies can boost [[saliva]] flow. Because [[Sjogren's syndrome]] increases your risk of [[dental]] cavities, limit sweets, especially between meals. Try artificial saliva. [[Saliva]] replacement products often work better than plain water because they contain a lubricant that helps your [[mouth]] stay moist longer. These products come as a spray or lozenge.

==References==
{{reflist|2}}

[[Category:Medicine]]
[[Category:Immunology]]
[[Category:Rheumatology]]
[[Category:Up-To-Date]]
[[Category:Primary care]]

Sjögren's syndrome overview

2020-05-10T18:20:11Z

Ayesha A. Khan: /* Secondary Prevention */

__NOTOC__
{{Sjögren's syndrome}}
{{CMG}} {{AE}} {{F.K}}

==Overview==
In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with enlargement of the [[parotid]] and [[lacrimal glands]] associated with a round-cell infiltrate and [[acinar]] [[atrophy]]. In 1933, Henrik Sjögren was the first to describe 19 females with clinical and pathological manifestations of the Sjögren's syndrome. American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to [[clinical]] findings. Common causes of Sjögren's syndrome include [[viral infection]] such as [[Epstein Barr virus|Epstein-Barr virus]] (EBV), [[Coxsackie virus]], [[Hepatitis C virus]], [[Cytomegalovirus]] (CMV), [[Human herpesvirus 6]] (HHV-6), [[Retroviruses]], and [[genetic]] factors. The [[incidence]] of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The [[prevalence]] of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California. Common risk factors in the development of Sjögren's syndrome include family history of [[autoimmune diseases]], serological markers such as low [[complement]] levels and [[cryoglobulinemia]] and [[parotid gland enlargement]]. The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as [[ocular]] and [[oral]] dryness. Common complications of Sjögren's syndrome include [[blurred vision]] and [[corneal]] damage, [[optic neuritis]], and [[lymphoma]]. [[Prognosis]] is generally good and presence of low [[complement]] level is associated with a particularly poor [[prognosis]] among patients with Sjögren's syndrome. The most common symptoms of Sjögren's syndrome include [[ocular]] and [[oral]] symptoms. Patients with Sjögren's syndrome may have a positive history of [[rheumatoid arthritis]] (RA), [[systemic lupus erythematous]] (SLE) and [[non-Hodgkin B-cell lymphoma]]. Physical examination of patients with is usually remarkable for dryness of all [[mucous membranes]] such as [[mouth]], [[eyes]], [[lips]], [[anal]] and [[rectal]]. Laboratory findings consistent with the diagnosis of Sjögren's syndrome include elevated [[erythrocyte sedimentation rate]] ([[Erythrocyte sedimentation rate|ESR]]), [[cytopenia]], the presence of anti-SSA/Ro, anti-SSB/La. Findings on an [[ultrasound]] suggestive of Sjögren's syndrome are hypoechoic and inhomogeneous [[salivary glands]], [[parenchymal]] inhomogeneity in the [[submandibular]] glands and focal or diffuse hypoechoic or anechoic foci in [[Gland|glands]]. [[Parotid gland]] [[Computed tomography|CT scan]] may be helpful in the diagnosis of Sjögren's syndrome and finding include abnormal [[diffuse]] [[Adipose tissue|fat tissue]] deposition and diffuse punctate [[calcification]]. The most commonly used tests for [[dry eyes]] of Sjögren's syndrome include [[Schirmer's test]], [[ocular]] surface staining and tear break-up time. Pharmacologic medical therapies for [[Keratoconjunctivitis sicca|dry eye]], [[Xerostomia|dry mouth]], and other sicca symptoms of Sjögren's syndrome are [[pilocarpine]], [[cevimeline]] and [[artificial tears]]. The mainstay of treatment for Sjögren's syndrome is medical therapy. Surgery is usually reserved for patients with [[occlusion]] of the lacrimal puncta, [[salivary gland]] [[malignancy]] and recurrent [[parotitis]] refractory to medical management.

==Historical Perspective==
In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with with enlargement of the [[parotid]] and [[lacrimal glands]] associated with a round-cell infiltrate and [[acinar]] [[atrophy]]. In 1933, Henrik Sjögren was the first to describe 19 females with clinical and [[Pathology (disambiguation)|pathological]] manifestations of the Sjögren's syndrome.

==Classification==
American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to [[clinical]] findings.

==Pathophysiology==
[[Sjögren's syndrome]] (SS) is a chronic [[Autoimmunity|autoimmune disorder]] that can affect several [[Organ (anatomy)|organ systems]]. [[Sjögren's syndrome]] is classified into a "primary" form that is a separate entity from other well-defined [[Autoimmune disease|autoimmune]] disorders and a "secondary" form that is associated with other well-defined [[Autoimmunity|autoimmune]] conditions, such as [[SLE]], [[rheumatoid arthritis]], [[Progressive Systemic Sclerosis|progressive systemic sclerosis]], and [[primary biliary cirrhosis]]. These forms of [[Sjögren's syndrome]] are different in their [[Serology|serologic]] and [[Histopathology|histopathologic]] findings as well as their [[Genetics|genetic]] components. Both [[Genetics|genetic]] and immune factors contribute to the [[pathogenesis]] of the disease. In the most common presentation of [[Sjögren's syndrome]], [[Lymphocyte|lymphocytes]] infiltrate the [[lacrimal]] and [[Salivary gland|salivary glands]] and impair their function, hence causing the main characteristic [[Symptom|symptoms]] such as [[Xerostomia|dry mouth]] ([[xerostomia]]) and [[dry eyes]] ([[keratoconjunctivitis sicca]]). [[CD4|CD4+]] [[T cell|T-cells]] are predominant in mild and moderate [[salivary gland]] infiltrations, while [[B cell|B cells]] play the major role in severe lesions. [[Sjögren's syndrome]] may also manifest itself with dryness of [[skin]] and other [[Mucous membrane|mucosal surfaces]] or even cause systemic extraglandular disturbances such as [[arthritis]], [[vasculitis]], [[Kidney|renal]], [[Lung|pulmonary]], [[Hematopoiesis|hematopoietic]], and [[Neurology|neurologic]] involvement. In general, a combination of [[Lymphocyte|lymphocytic]] infiltration, [[B cell|B lymphocyte]] hyperreactivity, production of certain [[Autoantibody|autoantibodies]], [[Gene|genes]] mostly involved in the production of [[MHC]] molecules and certain [[Virus|viral infections]] which <nowiki/>are all linked to the [[pathogenesis]] of [[Sjögren's syndrome]].

==Causes==
Common causes of Sjögren's syndrome include [[Infection|viral infection]] such as [[Epstein Barr virus|Epstein-Barr virus]] (EBV), [[Coxsackie virus]], [[Hepatitis C virus]], [[Cytomegalovirus]] (CMV), [[Human herpesvirus 6]] (HHV-6), [[Retroviruses]] and [[genetic]] factors.

==Differentiating {{PAGENAME}} from Other Diseases==

==Epidemiology and Demographics==
The [[incidence]] of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The [[prevalence]] of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. The [[mortality rate]] of Sjögren's syndrome is approximately 1.38. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California.

==Risk Factors==
Common risk factors in the development of Sjögren's syndrome include family history of [[autoimmune diseases]], serological markers such as low [[complement]] levels and [[cryoglobulinemia]] and [[parotid gland enlargement]].

==Screening==
There is insufficient evidence to recommend routine screening for Sjögren's syndrome.

==Natural History, Complications, and Prognosis==
===Natural History===
The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as [[ocular]] and [[oral]] dryness.
===Complications===
Common complications of Sjögren's syndrome include [[blurred vision]] and [[corneal]] damage, [[optic neuritis]] and [[lymphoma]].
===Prognosis===
[[Prognosis]] is generally good and presence of low [[complement]] level is associated with a particularly poor [[prognosis]] among patients with Sjögren's syndrome.

==Diagnosis==
===Diagnostic Study of Choice===

===History and Symptoms===
The most common symptoms of Sjögren's syndrome include [[ocular]] and [[Mouth|oral]] symptoms. Patients with Sjögren's syndrome may have a positive history of [[rheumatoid arthritis]] ([[Rheumatoid arthritis|RA]]), [[systemic lupus erythematous]] ([[SLE]]) and [[Non-Hodgkin lymphoma|non-Hodgkin B-cell lymphoma]].

===Physical Examination===
Physical examination of patients with is usually remarkable for dryness of all [[mucous membranes]] such as [[mouth]], [[Eye|eyes]], lips, [[anal]], and [[rectal]].

===Laboratory Findings===
Laboratory findings consistent with the diagnosis of Sjögren's syndrome include elevated [[erythrocyte sedimentation rate]] ([[Erythrocyte sedimentation rate|ESR]]), [[cytopenia]], presence of anti-SSA/Ro, and anti-SSB/La.

===Electrocardiogram===
There are no ECG findings associated with Sjögren's syndrome.

=== X-ray ===

=== Echocardiography and Ultrasound ===
Findings on an [[ultrasound]] suggestive of Sjögren's syndrome are hypoechoic and inhomogeneous [[salivary glands]], [[parenchymal]] inhomogeneity in the [[submandibular]] glands and focal or diffuse hypoechoic or anechoic foci in glands. There are no [[echocardiography]] findings associated with Sjögren's syndrome.

=== CT scan ===
[[Parotid gland]] [[Computed tomography|CT scan]] may be helpful in the diagnosis of Sjögren's syndrome and finding include abnormal diffuse [[Adipose tissue|fat tissue]] deposition and diffuse punctate [[calcification]].

=== MRI ===
Findings on [[Magnetic resonance imaging|MRI]] suggestive of Sjögren's syndrome include loss of homogeneity kin signal intensity on T1-weighted [[MR]] images and fat saturation suppressed focal high-intensity areas on T1-weighted images.

=== Other Imaging Findings ===
There are no other imaging findings associated with Sjögren's syndrome.

=== Other Diagnostic Studies ===
The most commonly used tests for dry eyes of Sjögren's syndrome include [[Schirmer's test]], [[ocular]] surface staining and [[Tears|tear]] break-up time.

==Treatment==
===Medical Therapy===
Pharmacologic medical therapies for [[Keratoconjunctivitis sicca|dry eye]], [[Xerostomia|dry mouth]], and other sicca symptom of Sjögren's syndrome are [[pilocarpine]], [[cevimeline]] and [[artificial tears]].

===Surgery===
The mainstay of treatment for Sjögren's syndrome is medical therapy. Surgery is usually reserved for patients with [[occlusion]] of the lacrimal puncta, [[salivary gland]] [[malignancy]], and recurrent [[parotitis]] refractory to medical management.

===Primary Prevention===
[[Sjögren's syndrome]] is [[inherited]] condition, there is no particular way to prevent developing the disease.

=== Secondary Prevention ===
Secondary prevention by Smoking cessation. Smoking can irritate and dry out your mouth. Increase your fluid intake. Take sips of fluids, particularly water, throughout the day. Avoid drinking coffee or alcohol since they can worsen dry mouth symptoms. Also avoid acidic beverages such as colas and some sports drinks because the acid can harm the enamel of your teeth. Stimulate saliva flow. Sugarless gum or citrus-flavored hard candies can boost saliva flow. Because Sjogren's syndrome increases your risk of dental cavities, limit sweets, especially between meals. Try artificial saliva. Saliva replacement products often work better than plain water because they contain a lubricant that helps your mouth stay moist longer. These products come as a spray or lozenge.

==References==
{{reflist|2}}

[[Category:Medicine]]
[[Category:Immunology]]
[[Category:Rheumatology]]
[[Category:Up-To-Date]]
[[Category:Primary care]]

Sjögren's syndrome overview

2020-05-10T18:11:49Z

Ayesha A. Khan: /* Primary Prevention */

__NOTOC__
{{Sjögren's syndrome}}
{{CMG}} {{AE}} {{F.K}}

==Overview==
In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with enlargement of the [[parotid]] and [[lacrimal glands]] associated with a round-cell infiltrate and [[acinar]] [[atrophy]]. In 1933, Henrik Sjögren was the first to describe 19 females with clinical and pathological manifestations of the Sjögren's syndrome. American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to [[clinical]] findings. Common causes of Sjögren's syndrome include [[viral infection]] such as [[Epstein Barr virus|Epstein-Barr virus]] (EBV), [[Coxsackie virus]], [[Hepatitis C virus]], [[Cytomegalovirus]] (CMV), [[Human herpesvirus 6]] (HHV-6), [[Retroviruses]], and [[genetic]] factors. The [[incidence]] of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The [[prevalence]] of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California. Common risk factors in the development of Sjögren's syndrome include family history of [[autoimmune diseases]], serological markers such as low [[complement]] levels and [[cryoglobulinemia]] and [[parotid gland enlargement]]. The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as [[ocular]] and [[oral]] dryness. Common complications of Sjögren's syndrome include [[blurred vision]] and [[corneal]] damage, [[optic neuritis]], and [[lymphoma]]. [[Prognosis]] is generally good and presence of low [[complement]] level is associated with a particularly poor [[prognosis]] among patients with Sjögren's syndrome. The most common symptoms of Sjögren's syndrome include [[ocular]] and [[oral]] symptoms. Patients with Sjögren's syndrome may have a positive history of [[rheumatoid arthritis]] (RA), [[systemic lupus erythematous]] (SLE) and [[non-Hodgkin B-cell lymphoma]]. Physical examination of patients with is usually remarkable for dryness of all [[mucous membranes]] such as [[mouth]], [[eyes]], [[lips]], [[anal]] and [[rectal]]. Laboratory findings consistent with the diagnosis of Sjögren's syndrome include elevated [[erythrocyte sedimentation rate]] ([[Erythrocyte sedimentation rate|ESR]]), [[cytopenia]], the presence of anti-SSA/Ro, anti-SSB/La. Findings on an [[ultrasound]] suggestive of Sjögren's syndrome are hypoechoic and inhomogeneous [[salivary glands]], [[parenchymal]] inhomogeneity in the [[submandibular]] glands and focal or diffuse hypoechoic or anechoic foci in [[Gland|glands]]. [[Parotid gland]] [[Computed tomography|CT scan]] may be helpful in the diagnosis of Sjögren's syndrome and finding include abnormal [[diffuse]] [[Adipose tissue|fat tissue]] deposition and diffuse punctate [[calcification]]. The most commonly used tests for [[dry eyes]] of Sjögren's syndrome include [[Schirmer's test]], [[ocular]] surface staining and tear break-up time. Pharmacologic medical therapies for [[Keratoconjunctivitis sicca|dry eye]], [[Xerostomia|dry mouth]], and other sicca symptoms of Sjögren's syndrome are [[pilocarpine]], [[cevimeline]] and [[artificial tears]]. The mainstay of treatment for Sjögren's syndrome is medical therapy. Surgery is usually reserved for patients with [[occlusion]] of the lacrimal puncta, [[salivary gland]] [[malignancy]] and recurrent [[parotitis]] refractory to medical management.

==Historical Perspective==
In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with with enlargement of the [[parotid]] and [[lacrimal glands]] associated with a round-cell infiltrate and [[acinar]] [[atrophy]]. In 1933, Henrik Sjögren was the first to describe 19 females with clinical and [[Pathology (disambiguation)|pathological]] manifestations of the Sjögren's syndrome.

==Classification==
American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to [[clinical]] findings.

==Pathophysiology==
[[Sjögren's syndrome]] (SS) is a chronic [[Autoimmunity|autoimmune disorder]] that can affect several [[Organ (anatomy)|organ systems]]. [[Sjögren's syndrome]] is classified into a "primary" form that is a separate entity from other well-defined [[Autoimmune disease|autoimmune]] disorders and a "secondary" form that is associated with other well-defined [[Autoimmunity|autoimmune]] conditions, such as [[SLE]], [[rheumatoid arthritis]], [[Progressive Systemic Sclerosis|progressive systemic sclerosis]], and [[primary biliary cirrhosis]]. These forms of [[Sjögren's syndrome]] are different in their [[Serology|serologic]] and [[Histopathology|histopathologic]] findings as well as their [[Genetics|genetic]] components. Both [[Genetics|genetic]] and immune factors contribute to the [[pathogenesis]] of the disease. In the most common presentation of [[Sjögren's syndrome]], [[Lymphocyte|lymphocytes]] infiltrate the [[lacrimal]] and [[Salivary gland|salivary glands]] and impair their function, hence causing the main characteristic [[Symptom|symptoms]] such as [[Xerostomia|dry mouth]] ([[xerostomia]]) and [[dry eyes]] ([[keratoconjunctivitis sicca]]). [[CD4|CD4+]] [[T cell|T-cells]] are predominant in mild and moderate [[salivary gland]] infiltrations, while [[B cell|B cells]] play the major role in severe lesions. [[Sjögren's syndrome]] may also manifest itself with dryness of [[skin]] and other [[Mucous membrane|mucosal surfaces]] or even cause systemic extraglandular disturbances such as [[arthritis]], [[vasculitis]], [[Kidney|renal]], [[Lung|pulmonary]], [[Hematopoiesis|hematopoietic]], and [[Neurology|neurologic]] involvement. In general, a combination of [[Lymphocyte|lymphocytic]] infiltration, [[B cell|B lymphocyte]] hyperreactivity, production of certain [[Autoantibody|autoantibodies]], [[Gene|genes]] mostly involved in the production of [[MHC]] molecules and certain [[Virus|viral infections]] which <nowiki/>are all linked to the [[pathogenesis]] of [[Sjögren's syndrome]].

==Causes==
Common causes of Sjögren's syndrome include [[Infection|viral infection]] such as [[Epstein Barr virus|Epstein-Barr virus]] (EBV), [[Coxsackie virus]], [[Hepatitis C virus]], [[Cytomegalovirus]] (CMV), [[Human herpesvirus 6]] (HHV-6), [[Retroviruses]] and [[genetic]] factors.

==Differentiating {{PAGENAME}} from Other Diseases==

==Epidemiology and Demographics==
The [[incidence]] of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The [[prevalence]] of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. The [[mortality rate]] of Sjögren's syndrome is approximately 1.38. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California.

==Risk Factors==
Common risk factors in the development of Sjögren's syndrome include family history of [[autoimmune diseases]], serological markers such as low [[complement]] levels and [[cryoglobulinemia]] and [[parotid gland enlargement]].

==Screening==
There is insufficient evidence to recommend routine screening for Sjögren's syndrome.

==Natural History, Complications, and Prognosis==
===Natural History===
The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as [[ocular]] and [[oral]] dryness.
===Complications===
Common complications of Sjögren's syndrome include [[blurred vision]] and [[corneal]] damage, [[optic neuritis]] and [[lymphoma]].
===Prognosis===
[[Prognosis]] is generally good and presence of low [[complement]] level is associated with a particularly poor [[prognosis]] among patients with Sjögren's syndrome.

==Diagnosis==
===Diagnostic Study of Choice===

===History and Symptoms===
The most common symptoms of Sjögren's syndrome include [[ocular]] and [[Mouth|oral]] symptoms. Patients with Sjögren's syndrome may have a positive history of [[rheumatoid arthritis]] ([[Rheumatoid arthritis|RA]]), [[systemic lupus erythematous]] ([[SLE]]) and [[Non-Hodgkin lymphoma|non-Hodgkin B-cell lymphoma]].

===Physical Examination===
Physical examination of patients with is usually remarkable for dryness of all [[mucous membranes]] such as [[mouth]], [[Eye|eyes]], lips, [[anal]], and [[rectal]].

===Laboratory Findings===
Laboratory findings consistent with the diagnosis of Sjögren's syndrome include elevated [[erythrocyte sedimentation rate]] ([[Erythrocyte sedimentation rate|ESR]]), [[cytopenia]], presence of anti-SSA/Ro, and anti-SSB/La.

===Electrocardiogram===
There are no ECG findings associated with Sjögren's syndrome.

=== X-ray ===

=== Echocardiography and Ultrasound ===
Findings on an [[ultrasound]] suggestive of Sjögren's syndrome are hypoechoic and inhomogeneous [[salivary glands]], [[parenchymal]] inhomogeneity in the [[submandibular]] glands and focal or diffuse hypoechoic or anechoic foci in glands. There are no [[echocardiography]] findings associated with Sjögren's syndrome.

=== CT scan ===
[[Parotid gland]] [[Computed tomography|CT scan]] may be helpful in the diagnosis of Sjögren's syndrome and finding include abnormal diffuse [[Adipose tissue|fat tissue]] deposition and diffuse punctate [[calcification]].

=== MRI ===
Findings on [[Magnetic resonance imaging|MRI]] suggestive of Sjögren's syndrome include loss of homogeneity kin signal intensity on T1-weighted [[MR]] images and fat saturation suppressed focal high-intensity areas on T1-weighted images.

=== Other Imaging Findings ===
There are no other imaging findings associated with Sjögren's syndrome.

=== Other Diagnostic Studies ===
The most commonly used tests for dry eyes of Sjögren's syndrome include [[Schirmer's test]], [[ocular]] surface staining and [[Tears|tear]] break-up time.

==Treatment==
===Medical Therapy===
Pharmacologic medical therapies for [[Keratoconjunctivitis sicca|dry eye]], [[Xerostomia|dry mouth]], and other sicca symptom of Sjögren's syndrome are [[pilocarpine]], [[cevimeline]] and [[artificial tears]].

===Surgery===
The mainstay of treatment for Sjögren's syndrome is medical therapy. Surgery is usually reserved for patients with [[occlusion]] of the lacrimal puncta, [[salivary gland]] [[malignancy]], and recurrent [[parotitis]] refractory to medical management.

===Primary Prevention===
[[Sjögren's syndrome]] is [[inherited]] condition, there is no particular way to prevent developing the disease.

=== Secondary Prevention ===

==References==
{{reflist|2}}

[[Category:Medicine]]
[[Category:Immunology]]
[[Category:Rheumatology]]
[[Category:Up-To-Date]]
[[Category:Primary care]]

Sjögren's syndrome overview

2020-05-10T18:11:19Z

Ayesha A. Khan: /* Primary Prevention */

__NOTOC__
{{Sjögren's syndrome}}
{{CMG}} {{AE}} {{F.K}}

==Overview==
In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with enlargement of the [[parotid]] and [[lacrimal glands]] associated with a round-cell infiltrate and [[acinar]] [[atrophy]]. In 1933, Henrik Sjögren was the first to describe 19 females with clinical and pathological manifestations of the Sjögren's syndrome. American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to [[clinical]] findings. Common causes of Sjögren's syndrome include [[viral infection]] such as [[Epstein Barr virus|Epstein-Barr virus]] (EBV), [[Coxsackie virus]], [[Hepatitis C virus]], [[Cytomegalovirus]] (CMV), [[Human herpesvirus 6]] (HHV-6), [[Retroviruses]], and [[genetic]] factors. The [[incidence]] of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The [[prevalence]] of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California. Common risk factors in the development of Sjögren's syndrome include family history of [[autoimmune diseases]], serological markers such as low [[complement]] levels and [[cryoglobulinemia]] and [[parotid gland enlargement]]. The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as [[ocular]] and [[oral]] dryness. Common complications of Sjögren's syndrome include [[blurred vision]] and [[corneal]] damage, [[optic neuritis]], and [[lymphoma]]. [[Prognosis]] is generally good and presence of low [[complement]] level is associated with a particularly poor [[prognosis]] among patients with Sjögren's syndrome. The most common symptoms of Sjögren's syndrome include [[ocular]] and [[oral]] symptoms. Patients with Sjögren's syndrome may have a positive history of [[rheumatoid arthritis]] (RA), [[systemic lupus erythematous]] (SLE) and [[non-Hodgkin B-cell lymphoma]]. Physical examination of patients with is usually remarkable for dryness of all [[mucous membranes]] such as [[mouth]], [[eyes]], [[lips]], [[anal]] and [[rectal]]. Laboratory findings consistent with the diagnosis of Sjögren's syndrome include elevated [[erythrocyte sedimentation rate]] ([[Erythrocyte sedimentation rate|ESR]]), [[cytopenia]], the presence of anti-SSA/Ro, anti-SSB/La. Findings on an [[ultrasound]] suggestive of Sjögren's syndrome are hypoechoic and inhomogeneous [[salivary glands]], [[parenchymal]] inhomogeneity in the [[submandibular]] glands and focal or diffuse hypoechoic or anechoic foci in [[Gland|glands]]. [[Parotid gland]] [[Computed tomography|CT scan]] may be helpful in the diagnosis of Sjögren's syndrome and finding include abnormal [[diffuse]] [[Adipose tissue|fat tissue]] deposition and diffuse punctate [[calcification]]. The most commonly used tests for [[dry eyes]] of Sjögren's syndrome include [[Schirmer's test]], [[ocular]] surface staining and tear break-up time. Pharmacologic medical therapies for [[Keratoconjunctivitis sicca|dry eye]], [[Xerostomia|dry mouth]], and other sicca symptoms of Sjögren's syndrome are [[pilocarpine]], [[cevimeline]] and [[artificial tears]]. The mainstay of treatment for Sjögren's syndrome is medical therapy. Surgery is usually reserved for patients with [[occlusion]] of the lacrimal puncta, [[salivary gland]] [[malignancy]] and recurrent [[parotitis]] refractory to medical management.

==Historical Perspective==
In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with with enlargement of the [[parotid]] and [[lacrimal glands]] associated with a round-cell infiltrate and [[acinar]] [[atrophy]]. In 1933, Henrik Sjögren was the first to describe 19 females with clinical and [[Pathology (disambiguation)|pathological]] manifestations of the Sjögren's syndrome.

==Classification==
American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to [[clinical]] findings.

==Pathophysiology==
[[Sjögren's syndrome]] (SS) is a chronic [[Autoimmunity|autoimmune disorder]] that can affect several [[Organ (anatomy)|organ systems]]. [[Sjögren's syndrome]] is classified into a "primary" form that is a separate entity from other well-defined [[Autoimmune disease|autoimmune]] disorders and a "secondary" form that is associated with other well-defined [[Autoimmunity|autoimmune]] conditions, such as [[SLE]], [[rheumatoid arthritis]], [[Progressive Systemic Sclerosis|progressive systemic sclerosis]], and [[primary biliary cirrhosis]]. These forms of [[Sjögren's syndrome]] are different in their [[Serology|serologic]] and [[Histopathology|histopathologic]] findings as well as their [[Genetics|genetic]] components. Both [[Genetics|genetic]] and immune factors contribute to the [[pathogenesis]] of the disease. In the most common presentation of [[Sjögren's syndrome]], [[Lymphocyte|lymphocytes]] infiltrate the [[lacrimal]] and [[Salivary gland|salivary glands]] and impair their function, hence causing the main characteristic [[Symptom|symptoms]] such as [[Xerostomia|dry mouth]] ([[xerostomia]]) and [[dry eyes]] ([[keratoconjunctivitis sicca]]). [[CD4|CD4+]] [[T cell|T-cells]] are predominant in mild and moderate [[salivary gland]] infiltrations, while [[B cell|B cells]] play the major role in severe lesions. [[Sjögren's syndrome]] may also manifest itself with dryness of [[skin]] and other [[Mucous membrane|mucosal surfaces]] or even cause systemic extraglandular disturbances such as [[arthritis]], [[vasculitis]], [[Kidney|renal]], [[Lung|pulmonary]], [[Hematopoiesis|hematopoietic]], and [[Neurology|neurologic]] involvement. In general, a combination of [[Lymphocyte|lymphocytic]] infiltration, [[B cell|B lymphocyte]] hyperreactivity, production of certain [[Autoantibody|autoantibodies]], [[Gene|genes]] mostly involved in the production of [[MHC]] molecules and certain [[Virus|viral infections]] which <nowiki/>are all linked to the [[pathogenesis]] of [[Sjögren's syndrome]].

==Causes==
Common causes of Sjögren's syndrome include [[Infection|viral infection]] such as [[Epstein Barr virus|Epstein-Barr virus]] (EBV), [[Coxsackie virus]], [[Hepatitis C virus]], [[Cytomegalovirus]] (CMV), [[Human herpesvirus 6]] (HHV-6), [[Retroviruses]] and [[genetic]] factors.

==Differentiating {{PAGENAME}} from Other Diseases==

==Epidemiology and Demographics==
The [[incidence]] of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The [[prevalence]] of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. The [[mortality rate]] of Sjögren's syndrome is approximately 1.38. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California.

==Risk Factors==
Common risk factors in the development of Sjögren's syndrome include family history of [[autoimmune diseases]], serological markers such as low [[complement]] levels and [[cryoglobulinemia]] and [[parotid gland enlargement]].

==Screening==
There is insufficient evidence to recommend routine screening for Sjögren's syndrome.

==Natural History, Complications, and Prognosis==
===Natural History===
The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as [[ocular]] and [[oral]] dryness.
===Complications===
Common complications of Sjögren's syndrome include [[blurred vision]] and [[corneal]] damage, [[optic neuritis]] and [[lymphoma]].
===Prognosis===
[[Prognosis]] is generally good and presence of low [[complement]] level is associated with a particularly poor [[prognosis]] among patients with Sjögren's syndrome.

==Diagnosis==
===Diagnostic Study of Choice===

===History and Symptoms===
The most common symptoms of Sjögren's syndrome include [[ocular]] and [[Mouth|oral]] symptoms. Patients with Sjögren's syndrome may have a positive history of [[rheumatoid arthritis]] ([[Rheumatoid arthritis|RA]]), [[systemic lupus erythematous]] ([[SLE]]) and [[Non-Hodgkin lymphoma|non-Hodgkin B-cell lymphoma]].

===Physical Examination===
Physical examination of patients with is usually remarkable for dryness of all [[mucous membranes]] such as [[mouth]], [[Eye|eyes]], lips, [[anal]], and [[rectal]].

===Laboratory Findings===
Laboratory findings consistent with the diagnosis of Sjögren's syndrome include elevated [[erythrocyte sedimentation rate]] ([[Erythrocyte sedimentation rate|ESR]]), [[cytopenia]], presence of anti-SSA/Ro, and anti-SSB/La.

===Electrocardiogram===
There are no ECG findings associated with Sjögren's syndrome.

=== X-ray ===

=== Echocardiography and Ultrasound ===
Findings on an [[ultrasound]] suggestive of Sjögren's syndrome are hypoechoic and inhomogeneous [[salivary glands]], [[parenchymal]] inhomogeneity in the [[submandibular]] glands and focal or diffuse hypoechoic or anechoic foci in glands. There are no [[echocardiography]] findings associated with Sjögren's syndrome.

=== CT scan ===
[[Parotid gland]] [[Computed tomography|CT scan]] may be helpful in the diagnosis of Sjögren's syndrome and finding include abnormal diffuse [[Adipose tissue|fat tissue]] deposition and diffuse punctate [[calcification]].

=== MRI ===
Findings on [[Magnetic resonance imaging|MRI]] suggestive of Sjögren's syndrome include loss of homogeneity kin signal intensity on T1-weighted [[MR]] images and fat saturation suppressed focal high-intensity areas on T1-weighted images.

=== Other Imaging Findings ===
There are no other imaging findings associated with Sjögren's syndrome.

=== Other Diagnostic Studies ===
The most commonly used tests for dry eyes of Sjögren's syndrome include [[Schirmer's test]], [[ocular]] surface staining and [[Tears|tear]] break-up time.

==Treatment==
===Medical Therapy===
Pharmacologic medical therapies for [[Keratoconjunctivitis sicca|dry eye]], [[Xerostomia|dry mouth]], and other sicca symptom of Sjögren's syndrome are [[pilocarpine]], [[cevimeline]] and [[artificial tears]].

===Surgery===
The mainstay of treatment for Sjögren's syndrome is medical therapy. Surgery is usually reserved for patients with [[occlusion]] of the lacrimal puncta, [[salivary gland]] [[malignancy]], and recurrent [[parotitis]] refractory to medical management.

===Primary Prevention===
*[[Sjögren's syndrome]] is [[inherited]] condition, there is no particular way to prevent developing the disease.

=== Secondary Prevention ===

==References==
{{reflist|2}}

[[Category:Medicine]]
[[Category:Immunology]]
[[Category:Rheumatology]]
[[Category:Up-To-Date]]
[[Category:Primary care]]

Systemic lupus erythematosus overview

2020-05-10T17:56:20Z

Ayesha A. Khan:

{| class="infobox mw-collapsible" id="floatvideo" style="position: fixed; top: 65%; width:361px; right: 10px; margin: 0 0 0 0; border: 0; float: right;"
| Title
|-
|-
| {{#ev:youtube|https://https://www.youtube.com/watch?v=0junqD4BLH4|350}}
|-
|}
__NOTOC__
{{Systemic lupus erythematosus}}

{{CMG}}; {{AE}} {{MIR}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com] {{RT}}

==Overview==
Systemic lupus erythematosus (SLE) is an [[autoimmune]] disease that can involve almost all body organs. [[Lupus]] may be classified into several subtypes according to the clinical features including [[systemic lupus erythematosus]], [[cutaneous lupus erythematosus]], [[Drug-induced lupus erythematosus|drug-induced lupus]], and [[Neonatal lupus erythematosus|neonatal lupus]]. The progression of SLE involves the [[immune system]]. Almost all of the [[pathological]] manifestations of SLE are due to [[antibody]] formation and deposition of [[immune complexes]] in different organs of the body. When the [[immune complexes]] are formed, they deposit in different body tissues and [[vessels]], which may lead to [[complement]] activation and more organ damage. Other factors, including [[genetic]] factors, hormonal abnormalities, and environmental factors, also play a role in the pathogenesis of SLE. There are no established causes of systemic lupus erythematosus, but the most potent [[risk factor|risk factors]] in the development of SLE are known to be female sex, [[HLA]] [[genetic mutations]], African American, Asian, or non-Causcasian race, [[ultraviolet light]] exposure, and previous exposure to certain [[Infection|infections]]. SLE must be differentiated from other diseases that cause skin [[rash]], and [[arthritis]] such as [[rheumatoid arthritis]] (RA), [[mixed connective tissue disease]] (MCTD), [[systemic sclerosis]] (SSc), [[dermatomyositis]] (DM), [[polymyositis]] (PM), and other [[autoimmune diseases]]. Worldwide, the [[prevalence]] of [[systemic lupus erythematosus]] is 60 per 100,000 persons. SLE can cause numerous flare ups. SLE usually develops in the second and third decades of life, although it can present at any age. SLE usually begins with mild symptoms such as [[fatigue]], [[fever]], and skin [[rashes]]. Without treatment, the patient will develop symptoms of [[end organ damage]], which eventually leads to death in most cases. Common complications of systemic lupus erythematosus include [[dermatitis]], [[nephritis|nephritis,]] and [[arthritis]]. Most of these complications occur in [[chronic]] cases and lead to significant [[Disability|debilitation]]. The prognosis of systemic lupus erythematosus can vary. SLE can range from a [[benign]] illness to an extremely rapidly progressive disease that can lead to [[Fulminant|fulminant organ failure]] and death. Without treatment, systemic lupus erythematosus results in very high [[mortality rate]]. During the mid-20th century, the mortality rate of SLE was reported to be higher than 60%. SLE can be diagnosed based on SLICC criteria. The patient may have a positive history of familial [[lupus]], [[skin rashes]] (especially [[Photosensitivity|photosensitive]] [[skin rashes]]), [[arthritis]], and [[fatigue]], which may be suggestive of [[systemic lupus erythematosus]]. The most common symptoms of [[SLE]] include constitutional symptoms such as [[fatigue]], [[fever]], [[myalgia]], and [[Weight|weight changes]]. Other, organ-specific symptoms mostly occur with disease progression. [[SLE]] may show a variety of symptoms in different organs depending on the complications of the disease. The therapy for systemic lupus erythematosus (SLE) is targeted towards controlling disease activity and preventing organ damage. The choice of treatment for systemic lupus erythematosus (SLE) varies based on the severity of the disease and symptoms. Generally, all patients should be treated with [[hydroxychloroquine]]. Other [[Pharmacology|pharmacologic]] medical therapies for SLE include [[glucocorticoids]] like oral [[prednisone]] or [[Intravenous therapy|intravenous]] [[methylprednisolone]], [[Non-steroidal anti-inflammatory drug|NSAIDs]] like [[celecoxib]], and [[immunosuppressive therapy]] with [[mycophenolate]], [[cyclophosphamide]], or, particularly in severe cases, [[rituximab]]. Cutaneous lupus erythematosus (CLE), if present without the involvement of any other organ system, can be treated with [[Topical steroid|topical corticosteroids]]. Other organ-related complications of SLE should be treated separately.

==Historical perspective==
The word "[[Systemic lupus erythematosus|lupus]]" means wolf in Latin, which refers to the comparison between the bites of wolves and the destructive injuries caused by SLE. The history of [[lupus erythematosus]] can be divided into three periods: classical, neoclassical, and modern.<ref name="pmid6348430">{{cite journal |vauthors=Blotzer JW |title=Systemic lupus erythematosus I: historical aspects |journal=Md State Med J |volume=32 |issue=6 |pages=439–41 |year=1983 |pmid=6348430 |doi= |url=}}</ref> The classical period refers to the ancient history, when there was no exact definition of the [[disease]]. During the neoclassical [[lupus]] era, scientists determined the manifestations of lupus and to define the action of the disease. Modern history focuses on understanding the microscopic features and [[pathogenesis]] of [[SLE]].

==Classification==
SLE may be classified into several subtypes according to clinical features, including: [[systemic lupus erythematosus]], [[cutaneous lupus erythematosus]], [[Drug-induced lupus erythematosus|drug-induced lupus]], and [[Neonatal lupus erythematosus|neonatal lupus]]. [[Systemic lupus erythematosus]] ([[SLE]]) itself may be classified into several subtypes based on [[Dermatology|dermatologic]] manifestations or [[glomerulonephritis]]. [[SLE]] may be classified according to [[Dermatology|dermatologic]] manifestations into 4 subtypes: acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), chronic cutaneous lupus erythematosus (CCLE), and intermittent cutaneous lupus erythematosus (ICLE). SLE may be classified according to [[glomerulonephritis]] into 6 subtypes: minimal mesangial lupus nephritis (class I), mesangial proliferative lupus nephritis (class II), focal lupus nephritis (class III), diffuse lupus nephritis (class IV), lupus membranous nephropathy (class V), and advanced sclerosing lupus nephritis (class VI).

==Pathophysiology==
The pathophysiology of systemic lupus erythematosus involves the [[immune system]]. Other factors such as [[genetic]] factors, [[hormonal]] abnormalities, and environmental factors also play a role. The most important environmental factors involved in the [[pathogenesis]] of SLE include [[ultraviolet]] (UV) light and certain [[infections]]. The most important [[Gene|genes]] involved in the [[pathogenesis]] of SLE include [[HLA-DR2]], [[HLA-DR3]], [[HLA]] class 3, C1q, and [[IRF5|interferon (IFN) regulatory factor 5]]. The most prominent events involving [[immune]] abnormalities relate to persistent activation of [[B cells]] and [[Plasma cell|plasma cells]] that make [[Autoantibody|auto-antibodies]] during disease progression. [[Self-antigen]] dependent activation of autoreactive [[B cells]] and [[CD4 T cells]] in secondary [[lymphoid organs]], leads to production of [[pathogenic]] [[autoantibodies]] that, along with [[inflammatory]] [[cytokines]], promotes tissue injury in [[lupus]]. Antigen-presenting [[dendritic cells]] are necessary for [[adaptive immune cell]] activation, and contribute to [[inflammatory]] [[cytokine]] production. [[Autoantibodies]] in complexes with [[autoantibodies]] contribute to innate [[immune cell]] activation and [[cytokine]] production. [[Genetic]] predisposition is a requisite for aberrant immune system acivation, in the setting of environmental and stochastic events. Abbreviations: DC, dendritic cells; pDC, plasmacytoid dendritic cells.The disease developmental process begins with the release of microparticles and [[proinflammatory]] [[cytokines]] from the cells that are undergoing [[apoptosis]]. Due to excessive number of cells undergoing [[apoptosis]], the body is unable to clear these microparticles entirely, and they are presented to [[dendritic cells]] as [[antigens]]. [[Dendritic cells]] process these microparticles, mature, and present these as [[antigens]] to [[T-cells]]. [[T-cells]], microparticles, and [[proinflammatory]] [[cytokines]] themselves trigger [[B-cell]] activation and [[autoantibody]] production. As a result, body tissues lose their self-tolerance. The most prominent events involving [[hormonal]] abnormalities are due to [[prolactin]] and [[estrogen]]. On microscopic [[histopathological]] analysis, [[apoptotic]] [[Keratinocyte|keratinocytes]], [[vacuolization]] of the [[basement membrane]], and [[dermal]] [[mucin]] deposition are characteristic findings of SLE [[dermatitis]], and active or inactive [[Endocapillary proliferative glomerulonephritis|endocapillary]] or extracapillary segmental [[glomerulonephritis]] are characteristic findings of [[lupus nephritis]].

== Causes ==
There are no established causes for systemic lupus erythematosus. Common causes of systemic lupus erythematosus include [[genetic]] predisposition, [[Auto-immune disease|auto-immune diseases]], and use of drugs. Less common causes of systemic lupus erythematosus include environmental factors and exposure to [[Ultraviolet light|ultraviolet (UV) light.]]

==Differentiating Systemic lupus erythematosus from other diseases==
Systemic lupus erythematosus (SLE) must be differentiated from other diseases that cause skin [[rash]], [[arthritis]], positive [[autoimmune]] serology, [[weight loss]], [[Fever|fevers]] and [[chronic pain]], such as [[rheumatoid arthritis]] (RA), [[mixed connective tissue disease]] (MCTD), [[systemic sclerosis]] (SSc), [[dermatomyositis]] (DM), [[polymyositis]] (PM), and other [[autoimmune diseases]].

==Epidemiology and Demographics==
Worldwide, the [[prevalence]] of [[systemic lupus erythematosus]] is 60 per 100,000 persons. In North America, South America, Europe, and Asia, the incidence of systemic lupus erythematosus ranges from as low as 1 per 100,000 persons to as high as 20 per 100,000 persons, with an average prevalence of 12 per 100,000 persons. The overall [[mortality rate]] of lupus is very high, estimated at approximately 50,000 deaths per 100,000. Women are more commonly affected with [[systemic lupus erythematosus]] than men. [[Systemic lupus erythematosus]] flare ups are more prevalent in women. [[Systemic lupus erythematosus]] is more prevalent in the African and Asian populations.

==Risk Factors==
The most potent [[risk factor]] in the development of systemic lupus erythematosus is being female.<ref name="pmid16896282">{{cite journal |vauthors=Grimaldi CM |title=Sex and systemic lupus erythematosus: the role of the sex hormones estrogen and prolactin on the regulation of autoreactive B cells |journal=Curr Opin Rheumatol |volume=18 |issue=5 |pages=456–61 |year=2006 |pmid=16896282 |doi=10.1097/01.bor.0000240354.37927.dd |url=}}</ref> Other risk factors include [[HLA]] [[genetic mutations]], being African American, Asian, or non-Causcasian, and previous exposure to certain [[Infection|infections]].

==Screening==

According to the United States Preventive Services Task Force, screening for systemic lupus erythematosus is not recommended.
==Natural history, complications and prognosis==
===Natural History===
Systemic lupus erythematosus (SLE) is an [[autoimmune disease]]. SLE involves many flare ups. SLE usually develops in the second and third decades of life, though it can present any age, beginning with mild symptoms such as [[fatigue]], [[fever]], and skin [[rashes]]. Without treatment, the patient will develop symptoms of [[end organ damage]], which will eventually lead to death in most patients. The disease's course can be divided into 4 subcategories: developmental phase, preclinical phase, clinical phase, and comorbid complication phase.

===Complications===
Common complications of systemic lupus erythematosus include [[dermatitis]], [[nephritis]], and [[arthritis]]. Most of these complications occur as part of the [[chronic]] activity of the disease, and lead to the [[Disability|debilitating]] characteristics of the disease.

===Prognosis===
The prognosis of systemic lupus erythematosus ranges from a [[benign]] illness to an extremely rapid progressive disease that can lead to [[Fulminant|fulminant organ failure]] and death. Without treatment, systemic lupus erythematosus will result in a very high [[mortality rate]], with a report of higher than a 60% mortality rate during the mid-20th century. The presence of [[nephritis]] is associated with a particularly poor prognosis among patients with SLE; SLE is associated with a 10-year mortality of more than 50% among patients with [[nephritis]]. The recent increase in [[survival rate]] of patients and better prognoses may be due to increased disease recognition with more sensitive diagnostic tests, earlier diagnosis or treatment, the inclusion of milder cases, increasingly judicious therapy, and prompt treatment of complications. Although improvements in SLE diagnosis have led to better prognoses, the [[mortality rate]] among SLE patients is still 5 times higher than the normal population.

==Diagnosis==
===Diagnostic criteria===
Based on SLICC criteria, to be diagnosed with SLE, the patient should have either at least 4 of 17 criteria, including at least 1 of the 11 clinical criteria and one of the six [[Immunological|immunologic]] criteria (for each criterion, any bullet is considered 1 clinical criteria), or a biopsy-proven [[nephritis]] compatible with [[SLE]] in the presence of [[Antinuclear antibodies|antinuclear antibodies (ANA)]] or [[Double stranded DNA antibody|anti-double-stranded DNA]] (dsDNA) [[antibodies]].

===History and Symptoms===

A positive history of familial [[lupus]], [[skin rashes]] (especially [[Photosensitivity|photosensitive]] [[skin rashes]]), [[arthritis]], and [[fatigue]] may be suggestive of [[systemic lupus erythematosus]]. The most common symptoms of [[SLE]] include constitutional symptoms like [[fatigue]], [[fever]], [[myalgia]], and [[Weight|weight changes]]. Other organ-specific symptoms mostly occur with disease progression. [[SLE]] may show a variety of symptoms in different organs depending on its complications.

===Physical Examination===
In the earlier stages of the disease, patients appear well, while in the late stages of the disease, patients are clearly ill with multi-organ involvement. The patient may show a wide range of skin manifestations including [[urticaria]], [[Bullous|bullous lesions]], [[malar rash]], and scarring [[alopecia]]. The patient may develop nasal and [[oral ulcers]]. [[Arthritis]] may lead to a decreased [[range of motion]], joint effusion, and [[arthralgia]]. Neurological manifestations including [[psychosis]], [[cognitive impairment]], and [[hallucinations]], may also be present.

===Laboratory Findings===

Laboratory findings consistent with the diagnosis of systemic lupus erythematosus include [[autoantibody]] elevation of [[ANA]], [[anti-dsDNA antibody]], [[anti-SM antibody]], and [[antiphospholipid antibodies]], and a decrease in [[complement]] levels. Nonspecific laboratory findings include mild [[pancytopenia]], elevated levels of [[creatinine]] and [[proteinuria]] due to [[renal failure]] (secondary to [[nephritis]]), elevated levels of [[ESR]] and [[C-reactive protein|CRP]] as [[Acute phase reactant|acute phase reactants]], decreased level of [[Complement|complements]], and positive [[Coombs test|direct Coombs test]].

===Imaging Findings===

==== Electrocardiographic findings ====
The most important and prevalent [[ECG]] findings associated with systemic lupus erythematosus (SLE) include [[sinus tachycardia]], [[ST segment changes]], and [[Ventricular arrhythmias|ventricular conduction disturbances]]. Other [[ECG]] findings are related to late complications of SLE and depend on the complication.

==== X-ray ====
On X-ray imaging, systemic lupus erythematosus (SLE) may be characterized by different features depending on the complications that have occured. The most common characteristic findings of SLE in X-ray include a [[Thumbprinting|thumb printing sign]] in abdominal graphy, blunting of the [[costophrenic angle]] due to [[pleural effusion]], [[cardiomegaly]], [[hepatomegaly]], [[Osteoporosis|osteoprosis]], tenosinovitis, and other manifestations depending on the complications.

==== CT scan ====
On abdominal [[CT-scans|CT scan]], systemic lupus erythematosus (SLE) may be characterized by [[hepatosplenomegaly]], [[pancreatic]] parenchymal enlargement, and [[ascites]]. On cardiac [[CT-scans|CT scan]], SLE may be characterized by enhancement of the thickened [[pericardium]]. On brain [[Computed tomography|CT scan,]] SLE may be characterized by [[brain atrophy]], [[stroke]] patterns like [[Cortical area|cortical]] hypodensity, and increased [[attenuation]] of the [[Cerebral cortex|cortex]].

==== MRI ====
On [[MRI|abdominal MRI]], systemic lupus erythematosus (SLE) may be characterized by [[hepatomegaly]], [[Pancreas|pancreatic]] parenchymal enlargement, and hypervascularity of [[mesentery]]. On [[cardiac MRI]], SLE may be characterized by mitral leaflet thickening, pericardial thickness, and [[Pericardial effusion|pericardial effusions]]. On brain [[MRI]], SLE may be characterized by [[white matter]] [[lesion|lesions]], changes in [[blood circulation]] of the brain, and patchy areas of enhancement. On musculoskeletal [[MRI]], SLE may be characterized by [[intramuscular]] [[edema]], [[Tenosynovitis|proliferative tenosynovitis]], and [[bone marrow]] [[edema]].

==== Ultrasound and echocardiography ====
On abdominal [[ultrasound]], systemic lupus erythematosus (SLE) may present with [[hepatosplenomegaly]], [[ascites]], hyperecho-kidney tissue due to [[nephritis]], and, rarely, [[cholecystitis]]. On synovial [[ultrasound]], SLE may present with synovial effusions and [[synovitis]]. On echocardiography, SLE may present with decreased [[ejection fraction]], cardiac wall motion abnormality, [[Pericarditis|effusion pericarditis]], and valve leaflet thickening.

==== Other imaging findings ====
One other imaging modality that can be used for the diagnosis of systemic lupus erythematosus complications is the double-contrast technique for [[gastritis]] evaluation. Another imaging technique that can be helpful in the diagnosis of SLE complications, especially early manifestations, is the [[technetium-99m]] scan. The scan can be used in different ways, including [[Scintigraphy|bone scintigraphy]] and [[Bone scan|bone scans]] to evaluate early and late [[bone]] complications, and for early evaluation of other organ complications including [[cardiac]], [[Hepatobiliary disease|hepatobiliary]], and [[pulmonary]] complications.

==== Other diagnostic studies ====
Other diagnostic tests that can be used for diagnosis of complications include a [[barium swallow]] for [[stricture]] diagnosis, [[biopsies]] of the [[kidneys]] and the [[endometrium]] for further diagnosis of the degree of involvement, [[paracentesis]] to evaluate body effusions, and [[arthrocentesis]] to differentiate different causes of [[arthritis]].

==Treatment==
===Medical Therapy===

The mainstay of therapy for systemic lupus erythematosus (SLE) is controlling disease activity and preventing organ damage. The treatment choice for systemic lupus erythematosus (SLE) varies based on the severity of the disease and symptoms. Generally, all patients with any type of SLE manifestation should be treated with [[hydroxychloroquine]], <nowiki/>regardless of the level of their disease. Other [[Pharmacology|pharmacologic]] medical therapies for SLE include [[glucocorticoids]] like oral [[prednisone]] or [[Intravenous therapy|intravenous]] [[methylprednisolone]], [[Non-steroidal anti-inflammatory drug|NSAIDs]] like [[celecoxib]], and [[immunosuppressive therapy]] with [[mycophenolate]], [[cyclophosphamide]], or [[rituximab]], particularly in severe cases. Cutaneous lupus erythematosus (CLE), if presented separately without any other system involvement, can be treated with [[Topical steroid|topical corticosteroids]]. Other organ-related complications of SLE should be treated separately.

=== Surgery ===
Surgical intervention is not recommended for the management of systemic lupus erythematosus.

==Prevention==
===Primary Prevention===
There is no established method for prevention of systemic lupus erythematosus.

===Secondary Prevention===
Secondary prevention strategies following systemic lupus erythematosus include using [[aspirin]], [[ACE inhibitor|ACE inhibitors]], and [[statins]] to reduce [[Atherosclerotic disease|atherosclerotic diseases]] and participating in [[Cancer screening|cancer screenings]].

==References==
{{reflist|2}}
[[Category:Disease]]

[[Category:Dermatology]]
[[Category:Poxviruses]]
[[Category:Sexually transmitted diseases]]

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

User:Ayesha A. Khan

2020-05-10T17:54:13Z

Ayesha A. Khan:

[[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com]

Dr. Ayesha Khan, MD is an Internist in Cleveland, OH.

==Contact==
Email: Ayesha.khan@stvincentcharity.com
==Specialties==
Internist, Rheumatology

==Hospital affiliations==

*Kindred Hospital-Cleveland-Gateway
*Outreach Professional Services Inc
*St. Vincent Charity Medical Center

Systemic lupus erythematosus overview

2020-05-10T17:50:56Z

Ayesha A. Khan:

{| class="infobox mw-collapsible" id="floatvideo" style="position: fixed; top: 65%; width:361px; right: 10px; margin: 0 0 0 0; border: 0; float: right;"
| Title
|-
|-
| {{#ev:youtube|https://https://www.youtube.com/watch?v=0junqD4BLH4|350}}
|-
|}
__NOTOC__
{{Systemic lupus erythematosus}}

{{CMG}}; {{AE}} {{MIR}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]] {{RT}}

==Overview==
Systemic lupus erythematosus (SLE) is an [[autoimmune]] disease that can involve almost all body organs. [[Lupus]] may be classified into several subtypes according to the clinical features including [[systemic lupus erythematosus]], [[cutaneous lupus erythematosus]], [[Drug-induced lupus erythematosus|drug-induced lupus]], and [[Neonatal lupus erythematosus|neonatal lupus]]. The progression of SLE involves the [[immune system]]. Almost all of the [[pathological]] manifestations of SLE are due to [[antibody]] formation and deposition of [[immune complexes]] in different organs of the body. When the [[immune complexes]] are formed, they deposit in different body tissues and [[vessels]], which may lead to [[complement]] activation and more organ damage. Other factors, including [[genetic]] factors, hormonal abnormalities, and environmental factors, also play a role in the pathogenesis of SLE. There are no established causes of systemic lupus erythematosus, but the most potent [[risk factor|risk factors]] in the development of SLE are known to be female sex, [[HLA]] [[genetic mutations]], African American, Asian, or non-Causcasian race, [[ultraviolet light]] exposure, and previous exposure to certain [[Infection|infections]]. SLE must be differentiated from other diseases that cause skin [[rash]], and [[arthritis]] such as [[rheumatoid arthritis]] (RA), [[mixed connective tissue disease]] (MCTD), [[systemic sclerosis]] (SSc), [[dermatomyositis]] (DM), [[polymyositis]] (PM), and other [[autoimmune diseases]]. Worldwide, the [[prevalence]] of [[systemic lupus erythematosus]] is 60 per 100,000 persons. SLE can cause numerous flare ups. SLE usually develops in the second and third decades of life, although it can present at any age. SLE usually begins with mild symptoms such as [[fatigue]], [[fever]], and skin [[rashes]]. Without treatment, the patient will develop symptoms of [[end organ damage]], which eventually leads to death in most cases. Common complications of systemic lupus erythematosus include [[dermatitis]], [[nephritis|nephritis,]] and [[arthritis]]. Most of these complications occur in [[chronic]] cases and lead to significant [[Disability|debilitation]]. The prognosis of systemic lupus erythematosus can vary. SLE can range from a [[benign]] illness to an extremely rapidly progressive disease that can lead to [[Fulminant|fulminant organ failure]] and death. Without treatment, systemic lupus erythematosus results in very high [[mortality rate]]. During the mid-20th century, the mortality rate of SLE was reported to be higher than 60%. SLE can be diagnosed based on SLICC criteria. The patient may have a positive history of familial [[lupus]], [[skin rashes]] (especially [[Photosensitivity|photosensitive]] [[skin rashes]]), [[arthritis]], and [[fatigue]], which may be suggestive of [[systemic lupus erythematosus]]. The most common symptoms of [[SLE]] include constitutional symptoms such as [[fatigue]], [[fever]], [[myalgia]], and [[Weight|weight changes]]. Other, organ-specific symptoms mostly occur with disease progression. [[SLE]] may show a variety of symptoms in different organs depending on the complications of the disease. The therapy for systemic lupus erythematosus (SLE) is targeted towards controlling disease activity and preventing organ damage. The choice of treatment for systemic lupus erythematosus (SLE) varies based on the severity of the disease and symptoms. Generally, all patients should be treated with [[hydroxychloroquine]]. Other [[Pharmacology|pharmacologic]] medical therapies for SLE include [[glucocorticoids]] like oral [[prednisone]] or [[Intravenous therapy|intravenous]] [[methylprednisolone]], [[Non-steroidal anti-inflammatory drug|NSAIDs]] like [[celecoxib]], and [[immunosuppressive therapy]] with [[mycophenolate]], [[cyclophosphamide]], or, particularly in severe cases, [[rituximab]]. Cutaneous lupus erythematosus (CLE), if present without the involvement of any other organ system, can be treated with [[Topical steroid|topical corticosteroids]]. Other organ-related complications of SLE should be treated separately.

==Historical perspective==
The word "[[Systemic lupus erythematosus|lupus]]" means wolf in Latin, which refers to the comparison between the bites of wolves and the destructive injuries caused by SLE. The history of [[lupus erythematosus]] can be divided into three periods: classical, neoclassical, and modern.<ref name="pmid6348430">{{cite journal |vauthors=Blotzer JW |title=Systemic lupus erythematosus I: historical aspects |journal=Md State Med J |volume=32 |issue=6 |pages=439–41 |year=1983 |pmid=6348430 |doi= |url=}}</ref> The classical period refers to the ancient history, when there was no exact definition of the [[disease]]. During the neoclassical [[lupus]] era, scientists determined the manifestations of lupus and to define the action of the disease. Modern history focuses on understanding the microscopic features and [[pathogenesis]] of [[SLE]].

==Classification==
SLE may be classified into several subtypes according to clinical features, including: [[systemic lupus erythematosus]], [[cutaneous lupus erythematosus]], [[Drug-induced lupus erythematosus|drug-induced lupus]], and [[Neonatal lupus erythematosus|neonatal lupus]]. [[Systemic lupus erythematosus]] ([[SLE]]) itself may be classified into several subtypes based on [[Dermatology|dermatologic]] manifestations or [[glomerulonephritis]]. [[SLE]] may be classified according to [[Dermatology|dermatologic]] manifestations into 4 subtypes: acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), chronic cutaneous lupus erythematosus (CCLE), and intermittent cutaneous lupus erythematosus (ICLE). SLE may be classified according to [[glomerulonephritis]] into 6 subtypes: minimal mesangial lupus nephritis (class I), mesangial proliferative lupus nephritis (class II), focal lupus nephritis (class III), diffuse lupus nephritis (class IV), lupus membranous nephropathy (class V), and advanced sclerosing lupus nephritis (class VI).

==Pathophysiology==
The pathophysiology of systemic lupus erythematosus involves the [[immune system]]. Other factors such as [[genetic]] factors, [[hormonal]] abnormalities, and environmental factors also play a role. The most important environmental factors involved in the [[pathogenesis]] of SLE include [[ultraviolet]] (UV) light and certain [[infections]]. The most important [[Gene|genes]] involved in the [[pathogenesis]] of SLE include [[HLA-DR2]], [[HLA-DR3]], [[HLA]] class 3, C1q, and [[IRF5|interferon (IFN) regulatory factor 5]]. The most prominent events involving [[immune]] abnormalities relate to persistent activation of [[B cells]] and [[Plasma cell|plasma cells]] that make [[Autoantibody|auto-antibodies]] during disease progression. [[Self-antigen]] dependent activation of autoreactive [[B cells]] and [[CD4 T cells]] in secondary [[lymphoid organs]], leads to production of [[pathogenic]] [[autoantibodies]] that, along with [[inflammatory]] [[cytokines]], promotes tissue injury in [[lupus]]. Antigen-presenting [[dendritic cells]] are necessary for [[adaptive immune cell]] activation, and contribute to [[inflammatory]] [[cytokine]] production. [[Autoantibodies]] in complexes with [[autoantibodies]] contribute to innate [[immune cell]] activation and [[cytokine]] production. [[Genetic]] predisposition is a requisite for aberrant immune system acivation, in the setting of environmental and stochastic events. Abbreviations: DC, dendritic cells; pDC, plasmacytoid dendritic cells.The disease developmental process begins with the release of microparticles and [[proinflammatory]] [[cytokines]] from the cells that are undergoing [[apoptosis]]. Due to excessive number of cells undergoing [[apoptosis]], the body is unable to clear these microparticles entirely, and they are presented to [[dendritic cells]] as [[antigens]]. [[Dendritic cells]] process these microparticles, mature, and present these as [[antigens]] to [[T-cells]]. [[T-cells]], microparticles, and [[proinflammatory]] [[cytokines]] themselves trigger [[B-cell]] activation and [[autoantibody]] production. As a result, body tissues lose their self-tolerance. The most prominent events involving [[hormonal]] abnormalities are due to [[prolactin]] and [[estrogen]]. On microscopic [[histopathological]] analysis, [[apoptotic]] [[Keratinocyte|keratinocytes]], [[vacuolization]] of the [[basement membrane]], and [[dermal]] [[mucin]] deposition are characteristic findings of SLE [[dermatitis]], and active or inactive [[Endocapillary proliferative glomerulonephritis|endocapillary]] or extracapillary segmental [[glomerulonephritis]] are characteristic findings of [[lupus nephritis]].

== Causes ==
There are no established causes for systemic lupus erythematosus. Common causes of systemic lupus erythematosus include [[genetic]] predisposition, [[Auto-immune disease|auto-immune diseases]], and use of drugs. Less common causes of systemic lupus erythematosus include environmental factors and exposure to [[Ultraviolet light|ultraviolet (UV) light.]]

==Differentiating Systemic lupus erythematosus from other diseases==
Systemic lupus erythematosus (SLE) must be differentiated from other diseases that cause skin [[rash]], [[arthritis]], positive [[autoimmune]] serology, [[weight loss]], [[Fever|fevers]] and [[chronic pain]], such as [[rheumatoid arthritis]] (RA), [[mixed connective tissue disease]] (MCTD), [[systemic sclerosis]] (SSc), [[dermatomyositis]] (DM), [[polymyositis]] (PM), and other [[autoimmune diseases]].

==Epidemiology and Demographics==
Worldwide, the [[prevalence]] of [[systemic lupus erythematosus]] is 60 per 100,000 persons. In North America, South America, Europe, and Asia, the incidence of systemic lupus erythematosus ranges from as low as 1 per 100,000 persons to as high as 20 per 100,000 persons, with an average prevalence of 12 per 100,000 persons. The overall [[mortality rate]] of lupus is very high, estimated at approximately 50,000 deaths per 100,000. Women are more commonly affected with [[systemic lupus erythematosus]] than men. [[Systemic lupus erythematosus]] flare ups are more prevalent in women. [[Systemic lupus erythematosus]] is more prevalent in the African and Asian populations.

==Risk Factors==
The most potent [[risk factor]] in the development of systemic lupus erythematosus is being female.<ref name="pmid16896282">{{cite journal |vauthors=Grimaldi CM |title=Sex and systemic lupus erythematosus: the role of the sex hormones estrogen and prolactin on the regulation of autoreactive B cells |journal=Curr Opin Rheumatol |volume=18 |issue=5 |pages=456–61 |year=2006 |pmid=16896282 |doi=10.1097/01.bor.0000240354.37927.dd |url=}}</ref> Other risk factors include [[HLA]] [[genetic mutations]], being African American, Asian, or non-Causcasian, and previous exposure to certain [[Infection|infections]].

==Screening==

According to the United States Preventive Services Task Force, screening for systemic lupus erythematosus is not recommended.
==Natural history, complications and prognosis==
===Natural History===
Systemic lupus erythematosus (SLE) is an [[autoimmune disease]]. SLE involves many flare ups. SLE usually develops in the second and third decades of life, though it can present any age, beginning with mild symptoms such as [[fatigue]], [[fever]], and skin [[rashes]]. Without treatment, the patient will develop symptoms of [[end organ damage]], which will eventually lead to death in most patients. The disease's course can be divided into 4 subcategories: developmental phase, preclinical phase, clinical phase, and comorbid complication phase.

===Complications===
Common complications of systemic lupus erythematosus include [[dermatitis]], [[nephritis]], and [[arthritis]]. Most of these complications occur as part of the [[chronic]] activity of the disease, and lead to the [[Disability|debilitating]] characteristics of the disease.

===Prognosis===
The prognosis of systemic lupus erythematosus ranges from a [[benign]] illness to an extremely rapid progressive disease that can lead to [[Fulminant|fulminant organ failure]] and death. Without treatment, systemic lupus erythematosus will result in a very high [[mortality rate]], with a report of higher than a 60% mortality rate during the mid-20th century. The presence of [[nephritis]] is associated with a particularly poor prognosis among patients with SLE; SLE is associated with a 10-year mortality of more than 50% among patients with [[nephritis]]. The recent increase in [[survival rate]] of patients and better prognoses may be due to increased disease recognition with more sensitive diagnostic tests, earlier diagnosis or treatment, the inclusion of milder cases, increasingly judicious therapy, and prompt treatment of complications. Although improvements in SLE diagnosis have led to better prognoses, the [[mortality rate]] among SLE patients is still 5 times higher than the normal population.

==Diagnosis==
===Diagnostic criteria===
Based on SLICC criteria, to be diagnosed with SLE, the patient should have either at least 4 of 17 criteria, including at least 1 of the 11 clinical criteria and one of the six [[Immunological|immunologic]] criteria (for each criterion, any bullet is considered 1 clinical criteria), or a biopsy-proven [[nephritis]] compatible with [[SLE]] in the presence of [[Antinuclear antibodies|antinuclear antibodies (ANA)]] or [[Double stranded DNA antibody|anti-double-stranded DNA]] (dsDNA) [[antibodies]].

===History and Symptoms===

A positive history of familial [[lupus]], [[skin rashes]] (especially [[Photosensitivity|photosensitive]] [[skin rashes]]), [[arthritis]], and [[fatigue]] may be suggestive of [[systemic lupus erythematosus]]. The most common symptoms of [[SLE]] include constitutional symptoms like [[fatigue]], [[fever]], [[myalgia]], and [[Weight|weight changes]]. Other organ-specific symptoms mostly occur with disease progression. [[SLE]] may show a variety of symptoms in different organs depending on its complications.

===Physical Examination===
In the earlier stages of the disease, patients appear well, while in the late stages of the disease, patients are clearly ill with multi-organ involvement. The patient may show a wide range of skin manifestations including [[urticaria]], [[Bullous|bullous lesions]], [[malar rash]], and scarring [[alopecia]]. The patient may develop nasal and [[oral ulcers]]. [[Arthritis]] may lead to a decreased [[range of motion]], joint effusion, and [[arthralgia]]. Neurological manifestations including [[psychosis]], [[cognitive impairment]], and [[hallucinations]], may also be present.

===Laboratory Findings===

Laboratory findings consistent with the diagnosis of systemic lupus erythematosus include [[autoantibody]] elevation of [[ANA]], [[anti-dsDNA antibody]], [[anti-SM antibody]], and [[antiphospholipid antibodies]], and a decrease in [[complement]] levels. Nonspecific laboratory findings include mild [[pancytopenia]], elevated levels of [[creatinine]] and [[proteinuria]] due to [[renal failure]] (secondary to [[nephritis]]), elevated levels of [[ESR]] and [[C-reactive protein|CRP]] as [[Acute phase reactant|acute phase reactants]], decreased level of [[Complement|complements]], and positive [[Coombs test|direct Coombs test]].

===Imaging Findings===

==== Electrocardiographic findings ====
The most important and prevalent [[ECG]] findings associated with systemic lupus erythematosus (SLE) include [[sinus tachycardia]], [[ST segment changes]], and [[Ventricular arrhythmias|ventricular conduction disturbances]]. Other [[ECG]] findings are related to late complications of SLE and depend on the complication.

==== X-ray ====
On X-ray imaging, systemic lupus erythematosus (SLE) may be characterized by different features depending on the complications that have occured. The most common characteristic findings of SLE in X-ray include a [[Thumbprinting|thumb printing sign]] in abdominal graphy, blunting of the [[costophrenic angle]] due to [[pleural effusion]], [[cardiomegaly]], [[hepatomegaly]], [[Osteoporosis|osteoprosis]], tenosinovitis, and other manifestations depending on the complications.

==== CT scan ====
On abdominal [[CT-scans|CT scan]], systemic lupus erythematosus (SLE) may be characterized by [[hepatosplenomegaly]], [[pancreatic]] parenchymal enlargement, and [[ascites]]. On cardiac [[CT-scans|CT scan]], SLE may be characterized by enhancement of the thickened [[pericardium]]. On brain [[Computed tomography|CT scan,]] SLE may be characterized by [[brain atrophy]], [[stroke]] patterns like [[Cortical area|cortical]] hypodensity, and increased [[attenuation]] of the [[Cerebral cortex|cortex]].

==== MRI ====
On [[MRI|abdominal MRI]], systemic lupus erythematosus (SLE) may be characterized by [[hepatomegaly]], [[Pancreas|pancreatic]] parenchymal enlargement, and hypervascularity of [[mesentery]]. On [[cardiac MRI]], SLE may be characterized by mitral leaflet thickening, pericardial thickness, and [[Pericardial effusion|pericardial effusions]]. On brain [[MRI]], SLE may be characterized by [[white matter]] [[lesion|lesions]], changes in [[blood circulation]] of the brain, and patchy areas of enhancement. On musculoskeletal [[MRI]], SLE may be characterized by [[intramuscular]] [[edema]], [[Tenosynovitis|proliferative tenosynovitis]], and [[bone marrow]] [[edema]].

==== Ultrasound and echocardiography ====
On abdominal [[ultrasound]], systemic lupus erythematosus (SLE) may present with [[hepatosplenomegaly]], [[ascites]], hyperecho-kidney tissue due to [[nephritis]], and, rarely, [[cholecystitis]]. On synovial [[ultrasound]], SLE may present with synovial effusions and [[synovitis]]. On echocardiography, SLE may present with decreased [[ejection fraction]], cardiac wall motion abnormality, [[Pericarditis|effusion pericarditis]], and valve leaflet thickening.

==== Other imaging findings ====
One other imaging modality that can be used for the diagnosis of systemic lupus erythematosus complications is the double-contrast technique for [[gastritis]] evaluation. Another imaging technique that can be helpful in the diagnosis of SLE complications, especially early manifestations, is the [[technetium-99m]] scan. The scan can be used in different ways, including [[Scintigraphy|bone scintigraphy]] and [[Bone scan|bone scans]] to evaluate early and late [[bone]] complications, and for early evaluation of other organ complications including [[cardiac]], [[Hepatobiliary disease|hepatobiliary]], and [[pulmonary]] complications.

==== Other diagnostic studies ====
Other diagnostic tests that can be used for diagnosis of complications include a [[barium swallow]] for [[stricture]] diagnosis, [[biopsies]] of the [[kidneys]] and the [[endometrium]] for further diagnosis of the degree of involvement, [[paracentesis]] to evaluate body effusions, and [[arthrocentesis]] to differentiate different causes of [[arthritis]].

==Treatment==
===Medical Therapy===

The mainstay of therapy for systemic lupus erythematosus (SLE) is controlling disease activity and preventing organ damage. The treatment choice for systemic lupus erythematosus (SLE) varies based on the severity of the disease and symptoms. Generally, all patients with any type of SLE manifestation should be treated with [[hydroxychloroquine]], <nowiki/>regardless of the level of their disease. Other [[Pharmacology|pharmacologic]] medical therapies for SLE include [[glucocorticoids]] like oral [[prednisone]] or [[Intravenous therapy|intravenous]] [[methylprednisolone]], [[Non-steroidal anti-inflammatory drug|NSAIDs]] like [[celecoxib]], and [[immunosuppressive therapy]] with [[mycophenolate]], [[cyclophosphamide]], or [[rituximab]], particularly in severe cases. Cutaneous lupus erythematosus (CLE), if presented separately without any other system involvement, can be treated with [[Topical steroid|topical corticosteroids]]. Other organ-related complications of SLE should be treated separately.

=== Surgery ===
Surgical intervention is not recommended for the management of systemic lupus erythematosus.

==Prevention==
===Primary Prevention===
There is no established method for prevention of systemic lupus erythematosus.

===Secondary Prevention===
Secondary prevention strategies following systemic lupus erythematosus include using [[aspirin]], [[ACE inhibitor|ACE inhibitors]], and [[statins]] to reduce [[Atherosclerotic disease|atherosclerotic diseases]] and participating in [[Cancer screening|cancer screenings]].

==References==
{{reflist|2}}
[[Category:Disease]]

[[Category:Dermatology]]
[[Category:Poxviruses]]
[[Category:Sexually transmitted diseases]]

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

Systemic lupus erythematosus overview

2020-05-10T17:49:31Z

Ayesha A. Khan: /* Pathophysiology */

{| class="infobox mw-collapsible" id="floatvideo" style="position: fixed; top: 65%; width:361px; right: 10px; margin: 0 0 0 0; border: 0; float: right;"
| Title
|-
|-
| {{#ev:youtube|https://https://www.youtube.com/watch?v=0junqD4BLH4|350}}
|-
|}
__NOTOC__
{{Systemic lupus erythematosus}}

{{CMG}}; {{AE}} {{MIR}} {{RT}}

==Overview==
Systemic lupus erythematosus (SLE) is an [[autoimmune]] disease that can involve almost all body organs. [[Lupus]] may be classified into several subtypes according to the clinical features including [[systemic lupus erythematosus]], [[cutaneous lupus erythematosus]], [[Drug-induced lupus erythematosus|drug-induced lupus]], and [[Neonatal lupus erythematosus|neonatal lupus]]. The progression of SLE involves the [[immune system]]. Almost all of the [[pathological]] manifestations of SLE are due to [[antibody]] formation and deposition of [[immune complexes]] in different organs of the body. When the [[immune complexes]] are formed, they deposit in different body tissues and [[vessels]], which may lead to [[complement]] activation and more organ damage. Other factors, including [[genetic]] factors, hormonal abnormalities, and environmental factors, also play a role in the pathogenesis of SLE. There are no established causes of systemic lupus erythematosus, but the most potent [[risk factor|risk factors]] in the development of SLE are known to be female sex, [[HLA]] [[genetic mutations]], African American, Asian, or non-Causcasian race, [[ultraviolet light]] exposure, and previous exposure to certain [[Infection|infections]]. SLE must be differentiated from other diseases that cause skin [[rash]], and [[arthritis]] such as [[rheumatoid arthritis]] (RA), [[mixed connective tissue disease]] (MCTD), [[systemic sclerosis]] (SSc), [[dermatomyositis]] (DM), [[polymyositis]] (PM), and other [[autoimmune diseases]]. Worldwide, the [[prevalence]] of [[systemic lupus erythematosus]] is 60 per 100,000 persons. SLE can cause numerous flare ups. SLE usually develops in the second and third decades of life, although it can present at any age. SLE usually begins with mild symptoms such as [[fatigue]], [[fever]], and skin [[rashes]]. Without treatment, the patient will develop symptoms of [[end organ damage]], which eventually leads to death in most cases. Common complications of systemic lupus erythematosus include [[dermatitis]], [[nephritis|nephritis,]] and [[arthritis]]. Most of these complications occur in [[chronic]] cases and lead to significant [[Disability|debilitation]]. The prognosis of systemic lupus erythematosus can vary. SLE can range from a [[benign]] illness to an extremely rapidly progressive disease that can lead to [[Fulminant|fulminant organ failure]] and death. Without treatment, systemic lupus erythematosus results in very high [[mortality rate]]. During the mid-20th century, the mortality rate of SLE was reported to be higher than 60%. SLE can be diagnosed based on SLICC criteria. The patient may have a positive history of familial [[lupus]], [[skin rashes]] (especially [[Photosensitivity|photosensitive]] [[skin rashes]]), [[arthritis]], and [[fatigue]], which may be suggestive of [[systemic lupus erythematosus]]. The most common symptoms of [[SLE]] include constitutional symptoms such as [[fatigue]], [[fever]], [[myalgia]], and [[Weight|weight changes]]. Other, organ-specific symptoms mostly occur with disease progression. [[SLE]] may show a variety of symptoms in different organs depending on the complications of the disease. The therapy for systemic lupus erythematosus (SLE) is targeted towards controlling disease activity and preventing organ damage. The choice of treatment for systemic lupus erythematosus (SLE) varies based on the severity of the disease and symptoms. Generally, all patients should be treated with [[hydroxychloroquine]]. Other [[Pharmacology|pharmacologic]] medical therapies for SLE include [[glucocorticoids]] like oral [[prednisone]] or [[Intravenous therapy|intravenous]] [[methylprednisolone]], [[Non-steroidal anti-inflammatory drug|NSAIDs]] like [[celecoxib]], and [[immunosuppressive therapy]] with [[mycophenolate]], [[cyclophosphamide]], or, particularly in severe cases, [[rituximab]]. Cutaneous lupus erythematosus (CLE), if present without the involvement of any other organ system, can be treated with [[Topical steroid|topical corticosteroids]]. Other organ-related complications of SLE should be treated separately.

==Historical perspective==
The word "[[Systemic lupus erythematosus|lupus]]" means wolf in Latin, which refers to the comparison between the bites of wolves and the destructive injuries caused by SLE. The history of [[lupus erythematosus]] can be divided into three periods: classical, neoclassical, and modern.<ref name="pmid6348430">{{cite journal |vauthors=Blotzer JW |title=Systemic lupus erythematosus I: historical aspects |journal=Md State Med J |volume=32 |issue=6 |pages=439–41 |year=1983 |pmid=6348430 |doi= |url=}}</ref> The classical period refers to the ancient history, when there was no exact definition of the [[disease]]. During the neoclassical [[lupus]] era, scientists determined the manifestations of lupus and to define the action of the disease. Modern history focuses on understanding the microscopic features and [[pathogenesis]] of [[SLE]].

==Classification==
SLE may be classified into several subtypes according to clinical features, including: [[systemic lupus erythematosus]], [[cutaneous lupus erythematosus]], [[Drug-induced lupus erythematosus|drug-induced lupus]], and [[Neonatal lupus erythematosus|neonatal lupus]]. [[Systemic lupus erythematosus]] ([[SLE]]) itself may be classified into several subtypes based on [[Dermatology|dermatologic]] manifestations or [[glomerulonephritis]]. [[SLE]] may be classified according to [[Dermatology|dermatologic]] manifestations into 4 subtypes: acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), chronic cutaneous lupus erythematosus (CCLE), and intermittent cutaneous lupus erythematosus (ICLE). SLE may be classified according to [[glomerulonephritis]] into 6 subtypes: minimal mesangial lupus nephritis (class I), mesangial proliferative lupus nephritis (class II), focal lupus nephritis (class III), diffuse lupus nephritis (class IV), lupus membranous nephropathy (class V), and advanced sclerosing lupus nephritis (class VI).

==Pathophysiology==
The pathophysiology of systemic lupus erythematosus involves the [[immune system]]. Other factors such as [[genetic]] factors, [[hormonal]] abnormalities, and environmental factors also play a role. The most important environmental factors involved in the [[pathogenesis]] of SLE include [[ultraviolet]] (UV) light and certain [[infections]]. The most important [[Gene|genes]] involved in the [[pathogenesis]] of SLE include [[HLA-DR2]], [[HLA-DR3]], [[HLA]] class 3, C1q, and [[IRF5|interferon (IFN) regulatory factor 5]]. The most prominent events involving [[immune]] abnormalities relate to persistent activation of [[B cells]] and [[Plasma cell|plasma cells]] that make [[Autoantibody|auto-antibodies]] during disease progression. [[Self-antigen]] dependent activation of autoreactive [[B cells]] and [[CD4 T cells]] in secondary [[lymphoid organs]], leads to production of [[pathogenic]] [[autoantibodies]] that, along with [[inflammatory]] [[cytokines]], promotes tissue injury in [[lupus]]. Antigen-presenting [[dendritic cells]] are necessary for [[adaptive immune cell]] activation, and contribute to [[inflammatory]] [[cytokine]] production. [[Autoantibodies]] in complexes with [[autoantibodies]] contribute to innate [[immune cell]] activation and [[cytokine]] production. [[Genetic]] predisposition is a requisite for aberrant immune system acivation, in the setting of environmental and stochastic events. Abbreviations: DC, dendritic cells; pDC, plasmacytoid dendritic cells.The disease developmental process begins with the release of microparticles and [[proinflammatory]] [[cytokines]] from the cells that are undergoing [[apoptosis]]. Due to excessive number of cells undergoing [[apoptosis]], the body is unable to clear these microparticles entirely, and they are presented to [[dendritic cells]] as [[antigens]]. [[Dendritic cells]] process these microparticles, mature, and present these as [[antigens]] to [[T-cells]]. [[T-cells]], microparticles, and [[proinflammatory]] [[cytokines]] themselves trigger [[B-cell]] activation and [[autoantibody]] production. As a result, body tissues lose their self-tolerance. The most prominent events involving [[hormonal]] abnormalities are due to [[prolactin]] and [[estrogen]]. On microscopic [[histopathological]] analysis, [[apoptotic]] [[Keratinocyte|keratinocytes]], [[vacuolization]] of the [[basement membrane]], and [[dermal]] [[mucin]] deposition are characteristic findings of SLE [[dermatitis]], and active or inactive [[Endocapillary proliferative glomerulonephritis|endocapillary]] or extracapillary segmental [[glomerulonephritis]] are characteristic findings of [[lupus nephritis]].

== Causes ==
There are no established causes for systemic lupus erythematosus. Common causes of systemic lupus erythematosus include [[genetic]] predisposition, [[Auto-immune disease|auto-immune diseases]], and use of drugs. Less common causes of systemic lupus erythematosus include environmental factors and exposure to [[Ultraviolet light|ultraviolet (UV) light.]]

==Differentiating Systemic lupus erythematosus from other diseases==
Systemic lupus erythematosus (SLE) must be differentiated from other diseases that cause skin [[rash]], [[arthritis]], positive [[autoimmune]] serology, [[weight loss]], [[Fever|fevers]] and [[chronic pain]], such as [[rheumatoid arthritis]] (RA), [[mixed connective tissue disease]] (MCTD), [[systemic sclerosis]] (SSc), [[dermatomyositis]] (DM), [[polymyositis]] (PM), and other [[autoimmune diseases]].

==Epidemiology and Demographics==
Worldwide, the [[prevalence]] of [[systemic lupus erythematosus]] is 60 per 100,000 persons. In North America, South America, Europe, and Asia, the incidence of systemic lupus erythematosus ranges from as low as 1 per 100,000 persons to as high as 20 per 100,000 persons, with an average prevalence of 12 per 100,000 persons. The overall [[mortality rate]] of lupus is very high, estimated at approximately 50,000 deaths per 100,000. Women are more commonly affected with [[systemic lupus erythematosus]] than men. [[Systemic lupus erythematosus]] flare ups are more prevalent in women. [[Systemic lupus erythematosus]] is more prevalent in the African and Asian populations.

==Risk Factors==
The most potent [[risk factor]] in the development of systemic lupus erythematosus is being female.<ref name="pmid16896282">{{cite journal |vauthors=Grimaldi CM |title=Sex and systemic lupus erythematosus: the role of the sex hormones estrogen and prolactin on the regulation of autoreactive B cells |journal=Curr Opin Rheumatol |volume=18 |issue=5 |pages=456–61 |year=2006 |pmid=16896282 |doi=10.1097/01.bor.0000240354.37927.dd |url=}}</ref> Other risk factors include [[HLA]] [[genetic mutations]], being African American, Asian, or non-Causcasian, and previous exposure to certain [[Infection|infections]].

==Screening==

According to the United States Preventive Services Task Force, screening for systemic lupus erythematosus is not recommended.
==Natural history, complications and prognosis==
===Natural History===
Systemic lupus erythematosus (SLE) is an [[autoimmune disease]]. SLE involves many flare ups. SLE usually develops in the second and third decades of life, though it can present any age, beginning with mild symptoms such as [[fatigue]], [[fever]], and skin [[rashes]]. Without treatment, the patient will develop symptoms of [[end organ damage]], which will eventually lead to death in most patients. The disease's course can be divided into 4 subcategories: developmental phase, preclinical phase, clinical phase, and comorbid complication phase.

===Complications===
Common complications of systemic lupus erythematosus include [[dermatitis]], [[nephritis]], and [[arthritis]]. Most of these complications occur as part of the [[chronic]] activity of the disease, and lead to the [[Disability|debilitating]] characteristics of the disease.

===Prognosis===
The prognosis of systemic lupus erythematosus ranges from a [[benign]] illness to an extremely rapid progressive disease that can lead to [[Fulminant|fulminant organ failure]] and death. Without treatment, systemic lupus erythematosus will result in a very high [[mortality rate]], with a report of higher than a 60% mortality rate during the mid-20th century. The presence of [[nephritis]] is associated with a particularly poor prognosis among patients with SLE; SLE is associated with a 10-year mortality of more than 50% among patients with [[nephritis]]. The recent increase in [[survival rate]] of patients and better prognoses may be due to increased disease recognition with more sensitive diagnostic tests, earlier diagnosis or treatment, the inclusion of milder cases, increasingly judicious therapy, and prompt treatment of complications. Although improvements in SLE diagnosis have led to better prognoses, the [[mortality rate]] among SLE patients is still 5 times higher than the normal population.

==Diagnosis==
===Diagnostic criteria===
Based on SLICC criteria, to be diagnosed with SLE, the patient should have either at least 4 of 17 criteria, including at least 1 of the 11 clinical criteria and one of the six [[Immunological|immunologic]] criteria (for each criterion, any bullet is considered 1 clinical criteria), or a biopsy-proven [[nephritis]] compatible with [[SLE]] in the presence of [[Antinuclear antibodies|antinuclear antibodies (ANA)]] or [[Double stranded DNA antibody|anti-double-stranded DNA]] (dsDNA) [[antibodies]].

===History and Symptoms===

A positive history of familial [[lupus]], [[skin rashes]] (especially [[Photosensitivity|photosensitive]] [[skin rashes]]), [[arthritis]], and [[fatigue]] may be suggestive of [[systemic lupus erythematosus]]. The most common symptoms of [[SLE]] include constitutional symptoms like [[fatigue]], [[fever]], [[myalgia]], and [[Weight|weight changes]]. Other organ-specific symptoms mostly occur with disease progression. [[SLE]] may show a variety of symptoms in different organs depending on its complications.

===Physical Examination===
In the earlier stages of the disease, patients appear well, while in the late stages of the disease, patients are clearly ill with multi-organ involvement. The patient may show a wide range of skin manifestations including [[urticaria]], [[Bullous|bullous lesions]], [[malar rash]], and scarring [[alopecia]]. The patient may develop nasal and [[oral ulcers]]. [[Arthritis]] may lead to a decreased [[range of motion]], joint effusion, and [[arthralgia]]. Neurological manifestations including [[psychosis]], [[cognitive impairment]], and [[hallucinations]], may also be present.

===Laboratory Findings===

Laboratory findings consistent with the diagnosis of systemic lupus erythematosus include [[autoantibody]] elevation of [[ANA]], [[anti-dsDNA antibody]], [[anti-SM antibody]], and [[antiphospholipid antibodies]], and a decrease in [[complement]] levels. Nonspecific laboratory findings include mild [[pancytopenia]], elevated levels of [[creatinine]] and [[proteinuria]] due to [[renal failure]] (secondary to [[nephritis]]), elevated levels of [[ESR]] and [[C-reactive protein|CRP]] as [[Acute phase reactant|acute phase reactants]], decreased level of [[Complement|complements]], and positive [[Coombs test|direct Coombs test]].

===Imaging Findings===

==== Electrocardiographic findings ====
The most important and prevalent [[ECG]] findings associated with systemic lupus erythematosus (SLE) include [[sinus tachycardia]], [[ST segment changes]], and [[Ventricular arrhythmias|ventricular conduction disturbances]]. Other [[ECG]] findings are related to late complications of SLE and depend on the complication.

==== X-ray ====
On X-ray imaging, systemic lupus erythematosus (SLE) may be characterized by different features depending on the complications that have occured. The most common characteristic findings of SLE in X-ray include a [[Thumbprinting|thumb printing sign]] in abdominal graphy, blunting of the [[costophrenic angle]] due to [[pleural effusion]], [[cardiomegaly]], [[hepatomegaly]], [[Osteoporosis|osteoprosis]], tenosinovitis, and other manifestations depending on the complications.

==== CT scan ====
On abdominal [[CT-scans|CT scan]], systemic lupus erythematosus (SLE) may be characterized by [[hepatosplenomegaly]], [[pancreatic]] parenchymal enlargement, and [[ascites]]. On cardiac [[CT-scans|CT scan]], SLE may be characterized by enhancement of the thickened [[pericardium]]. On brain [[Computed tomography|CT scan,]] SLE may be characterized by [[brain atrophy]], [[stroke]] patterns like [[Cortical area|cortical]] hypodensity, and increased [[attenuation]] of the [[Cerebral cortex|cortex]].

==== MRI ====
On [[MRI|abdominal MRI]], systemic lupus erythematosus (SLE) may be characterized by [[hepatomegaly]], [[Pancreas|pancreatic]] parenchymal enlargement, and hypervascularity of [[mesentery]]. On [[cardiac MRI]], SLE may be characterized by mitral leaflet thickening, pericardial thickness, and [[Pericardial effusion|pericardial effusions]]. On brain [[MRI]], SLE may be characterized by [[white matter]] [[lesion|lesions]], changes in [[blood circulation]] of the brain, and patchy areas of enhancement. On musculoskeletal [[MRI]], SLE may be characterized by [[intramuscular]] [[edema]], [[Tenosynovitis|proliferative tenosynovitis]], and [[bone marrow]] [[edema]].

==== Ultrasound and echocardiography ====
On abdominal [[ultrasound]], systemic lupus erythematosus (SLE) may present with [[hepatosplenomegaly]], [[ascites]], hyperecho-kidney tissue due to [[nephritis]], and, rarely, [[cholecystitis]]. On synovial [[ultrasound]], SLE may present with synovial effusions and [[synovitis]]. On echocardiography, SLE may present with decreased [[ejection fraction]], cardiac wall motion abnormality, [[Pericarditis|effusion pericarditis]], and valve leaflet thickening.

==== Other imaging findings ====
One other imaging modality that can be used for the diagnosis of systemic lupus erythematosus complications is the double-contrast technique for [[gastritis]] evaluation. Another imaging technique that can be helpful in the diagnosis of SLE complications, especially early manifestations, is the [[technetium-99m]] scan. The scan can be used in different ways, including [[Scintigraphy|bone scintigraphy]] and [[Bone scan|bone scans]] to evaluate early and late [[bone]] complications, and for early evaluation of other organ complications including [[cardiac]], [[Hepatobiliary disease|hepatobiliary]], and [[pulmonary]] complications.

==== Other diagnostic studies ====
Other diagnostic tests that can be used for diagnosis of complications include a [[barium swallow]] for [[stricture]] diagnosis, [[biopsies]] of the [[kidneys]] and the [[endometrium]] for further diagnosis of the degree of involvement, [[paracentesis]] to evaluate body effusions, and [[arthrocentesis]] to differentiate different causes of [[arthritis]].

==Treatment==
===Medical Therapy===

The mainstay of therapy for systemic lupus erythematosus (SLE) is controlling disease activity and preventing organ damage. The treatment choice for systemic lupus erythematosus (SLE) varies based on the severity of the disease and symptoms. Generally, all patients with any type of SLE manifestation should be treated with [[hydroxychloroquine]], <nowiki/>regardless of the level of their disease. Other [[Pharmacology|pharmacologic]] medical therapies for SLE include [[glucocorticoids]] like oral [[prednisone]] or [[Intravenous therapy|intravenous]] [[methylprednisolone]], [[Non-steroidal anti-inflammatory drug|NSAIDs]] like [[celecoxib]], and [[immunosuppressive therapy]] with [[mycophenolate]], [[cyclophosphamide]], or [[rituximab]], particularly in severe cases. Cutaneous lupus erythematosus (CLE), if presented separately without any other system involvement, can be treated with [[Topical steroid|topical corticosteroids]]. Other organ-related complications of SLE should be treated separately.

=== Surgery ===
Surgical intervention is not recommended for the management of systemic lupus erythematosus.

==Prevention==
===Primary Prevention===
There is no established method for prevention of systemic lupus erythematosus.

===Secondary Prevention===
Secondary prevention strategies following systemic lupus erythematosus include using [[aspirin]], [[ACE inhibitor|ACE inhibitors]], and [[statins]] to reduce [[Atherosclerotic disease|atherosclerotic diseases]] and participating in [[Cancer screening|cancer screenings]].

==References==
{{reflist|2}}
[[Category:Disease]]

[[Category:Dermatology]]
[[Category:Poxviruses]]
[[Category:Sexually transmitted diseases]]

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

Rheumatoid arthritis overview

2020-05-10T17:39:12Z

Ayesha A. Khan:

__NOTOC__
{{Rheumatoid arthritis}}
{{CMG}} {{AE}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]], {{MKK}}

==Overview==
[[Rheumatoid arthritis]] is a chronic inflammatory systemic disease which is found commonly in women and starts in mid 30's to 40's. Cause of [[rheumatoid arthritis]] is idiopathic but there are [[Genetics|genetic]] factors such as [[HLA]]-DR4 cluster, [[Environmental Health Perspectives|environmental]] factors like [[smoking]], hormonal factors like [[hyperprolactinemia]], infectious factors like [[mycoplasma]], epstein-Barr virus ([[EBV]]), [[rubella virus]], [[porphyromonas gingivalis]], socioeconomic, [[psychological]], and lifestyle factors such as [[obesity]] play important role in the development of [[rheumatoid arthritis]]. There is no established system for the classification of [[rheumatoid arthritis]]. Depending on the presentation of [[rheumatoid arthritis]], it is classified into typical classic [[rheumatoid arthritis]] and palindromic [[rheumatism]]. Common symptoms of [[rheumatoid arthritis]] are [[joint pain]] of small joints of the hands, wrist, and forefoot, [[joint swelling]], morning [[joint stiffness]] classically lasting for more than an hour, decreased grip strength, generalized [[aching]], and [[fatigue]], [[weight loss]], [[depression]]. Less common symptoms are [[tingling]] and [[numbness]] in the arm and [[weakness]] in the arm. Anti-CCP antibodies testing is the gold standard test for the diagnosis of [[rheumatoid arthritis]].It has the specificity of about 81-100%, with the sensitivity of 39–94%. Hallmark of [[rheumatoid arthritis]] Xray findings are soft tissue swelling, joint space narrowing, and erosions but it involves small joints with multiple deformities. Findings on an ultrasound suggestive of [[rheumatoid arthritis]] are [[tenosynovitis]], [[bursitis]], [[baker's cyst]] and [[inflammation]] of [[synovium]]. Therapeutic use of [[ultrasound]] is the guidance of [[corticosteroid]] injection in the joint and fluid aspiration from the joint effusion. MRI is helpful in the diagnosis of early and subacute [[rheumatoid arthritis]]. Findings on MRI diagnostic of [[rheumatoid arthritis]] are [[synovial]] hyperemia, [[synovial]] [[hyperplasia]], [[pannus]], subchondral [[cysts]], erosions, effusion of [[joints]] and bone marrow [[edema]]. The mainstay of treatment of [[rheumatoid arthritis]] is pharmacotherapy. There are two types of therapies- non-pharmacological therapy and pharmacological therapy. There are different drug regimen depending upon the stage of the disease. In active stage, combined therapy include disease-modifying antirheumatic drugs ([[DMARDs]]) are used along with the [[anti-inflammatory drugs]]. Therapy for resistant disease and flares is the addition of oral [[glucocorticoids]]. In case of the flare, add another [[DMARDs]] with [[methotrexate]] or replace with another [[DMARDs]]. Surgery is usually reserved for patients with debilitating disease, failure of medical therapy and radiographic findings of end-stage [[arthritis]]. Various surgical options used for the treatment of [[RA]] are tenosynovectomy, [[arthroscopic]] or open [[synovectomy]], joint fusion, small joint implant [[arthroplasty]], total joint replacement of deformed joint. Effective measures for the primary prevention of [[rheumatoid arthritis]] are [[patient education]], [[exercise]], [[Physical Therapy|physical]] and [[occupational therapy]] and cognitive behavioral therapies. Effective measures for the secondary prevention of [[rheumatoid arthritis]] are [[calcium]] and [[vitamin D]] supplementation to prevent [[osteoporosis]]. To prevent [[cardiovascular]] complications and recurrent attacks of [[RA]], effective methods are [[exercise]], [[smoking]] cessation, and [[dietary control]].

==Historical Perspective==
[[Rheumatoid arthritis]] signs and symptoms were first noticed by Augustin Jacob Landre-Beauvais, a resident physician at the Saltpetriere asylum in France, in 1800, He named it as Goutte Asthenique Primitive or Primary Asthenic [[Gout]]. The name [[Rheumatoid arthritis]] coined by Archibald Garrod in 1890. In 1970, [[human leukocyte antigen]] (HLA) was first implicated in the pathogenesis of [[rheumatoid arthritis]].

==Classification==
There is no established system for the classification of [[rheumatoid arthritis]]. Depending on the presentation of [[rheumatoid arthritis]], it is classified into typical classic [[rheumatoid arthritis]] and palindromic [[rheumatism]]. The classification criterion for the diagnosis of rheumatoid arthritis according to American College of Rheumatology, uses 4 parameters including [[joint]] involvement, [[serology]], duration of [[symptoms]], and acute phase reactants. If the score is more than 6 according to this criteria, the disease is classified as definite [[rheumatoid arthritis]].

==Pathophysiology==
[[Rheumatoid arthritis]] is mediated by the combination of a predisposing [[genotype]] upon which [[genetic]] factors, [[Environmental Lung Diseases|environmental]] and [[Microorganisms|microorganism]] also contribute resulting in the [[inflammation]] and destruction of the [[synovial membrane]]. All the factors lead to [[citrullination]] or [[post-translational modifications]], the altered [[peptides]] bind to [[MHC]] protein with shared [[epitopes]] which further lead to [[antigen]] presentation to [[T-cells]].
[[T-cells]] further stimulate [[B-cells]] and cytokines which leads to cartilage damage. [[Mutation]] of [[human leukocyte antigen]] (HLA) genes on [[chromosome]] 6 is involved in pathogenesis of [[rheumatoid arthritis]]. Conditions associated with [[RA]] are [[vasculitis]], [[uveitis]], [[scleritis]], peripheral [[ulcerative keratitis]],
[[interstitial fibrosis]], pulmonary [[nodules]], [[bronchiolitis obliterans]], organizing [[pneumonia]], [[venous thromboembolism]], [[pericarditis]], [[myocarditis]], [[congestive heart failure]], [[atrial fibrillation]], [[sjogren's syndrome]], [[felty's syndrome]].

==Causes==
[[Rheumatoid arthritis]] occurs when your [[immune system]] attacks the [[synovium]] — the lining of the membranes that surround your [[joint]]s.The resulting [[inflammation]] thickens the [[synovium]], which can eventually destroy the [[cartilage]] and [[bone]] within the [[joint]].The [[tendon]]s and [[ligament]]s that hold the [[joint]] together weaken and stretch. Gradually, the joint loses its shape and alignment.Cause of [[rheumatoid arthritis]] is idiopathic but [[Genetics|genetic]] like [[HLA]]-DR4 cluster, [[Environmental Health Perspectives|environmental]] like [[smoking]], hormonal like [[hyperprolactinemia]], [[Immunological|immunologic]], infectious factors like [[mycoplasma]], epstein-Barr virus ([[EBV]]), [[rubella virus]], [[porphyromonas gingivalis]], socioeconomic, [[psychological]], and lifestyle factors such as [[obesity]] play important role in the development of [[rheumatoid arthritis]].

==Differentiating Rheumatoid Arthritis from other Diseases==
[[Rheumatoid arthritis]] must be differentiated from [[osteoarthritis]] , [[septic arthritis]], [[reactive arthritis]], [[Behçet's disease|behcet's disease]] and [[psoriatic arthritis]].

==Epidemiology and Demographics==
The incidence of [[rheumatoid arthritis]] is approximately 40 per 100,000 individuals worldwide. The prevalence of [[rheumatoid arthritis]] is approximately 1 percent in Caucasians per 100,000 individuals worldwide.The peak onset of the disease is between the age of 50 and 75 years. Women are three times more commonly affected by [[rheumatoid arthritis]] than men. The mortality rate of [[rheumatoid arthritis]] is approximately 10%.

==Risk Factors==
Common risk factors in the development of [[rheumatoid arthritis]] are the family history of [[RA]], cigarette [[smoking]], race like more prevalent in Native Americans, strongly associated with [[major histocompatibility complex]] (MHC) class II antigen [[human leukocyte antigen|HLA]]-DR4 and patient with [[silica]] and [[asbestos]] exposure.
Less common risk factors are [[obesity]] and high consumption of red meat.

==Screening==
According to the American College of Rheumatology Guideline, screening for [[rheumatoid arthritis]] is not recommended.

==Natural History, Complications and Prognosis==
The symptoms of [[rheumatoid arthritis]] usually develop in the third to fourth decade of life and start with symptoms such as fatigue, small joints pain and morning stiffness which lasts more than 1 hour. Complication of [[rheumatoid arthritis]] involve all the systems. Cardiac complications are pericarditis, congestive heart failure, endocarditis and myocardial infarction. Pulmonary complications are pleurisy, [[alveolitis]], [[pleural effusion]] and [[pulmonary fibrosis]]. Rheumatological complications are [[joint]] deformities, [[felty's syndrome]], [[sjögren's syndrome]], [[osteoporosis]] and atlantoaxial subluxation. Eye complications such as [[scleritis]] and [[keratitis]]. Renal complications are [[chronic renal failure]] from [[amyloidosis]]. Nervous system complications like [[peripheral neuropathy]] and [[mononeuritis multiplex]]. Prognosis is generally good with early diagnosis and treatment, and 10-year disability rate of patients with rheumatoid arthritis is approximately 40%.

==Diagnostic study of choice==
Anti-CCP antibodies testing is the gold standard test for the diagnosis of [[rheumatoid arthritis]].It has the specificity of about 81-100%, with the [[sensitivity]] of 39–94%.

==History and Symptoms==
Patients with [[rheumatoid arthritis]] may have a positive history of the family history of [[RA]], [[history of smoking]] and other [[autoimmune disease]]. Common symptoms of [[rheumatoid arthritis]] are [[joint pain]] of small joints of the hands, wrist, and forefoot, [[joint swelling]], morning [[joint stiffness]] classically lasting for more than an hour, decreased grip strength, generalized [[aching]], and [[fatigue]], [[weight loss]], [[depression]]. Less common symptoms are [[tingling]] and [[numbness]] in the arm and [[weakness]] in the arm.

==Physical Examination==
Patients with rheumatoid arthritis usually appear fatigued. On skin examination, [[Rheumatoid nodule|rheumatoid nodules]], [[erythema nodosum]], [[atrophy]] of the digital skin, [[palmar erythema]] and diffuse thinning of the skin are found. If there is involvement of eyes, [[Scleritis]] and [[Scleromalacia]] are seen. On auscultation of lungs, there are decreased breath sounds on both sides and [[crackles]] may be present. On palpation of the abdomen, [[hepatomegaly]] and [[splenomegaly]] is usually found when it is associated with [[Felty's syndrome]]. On examination of extremities, [[redness]], and swelling of the affected joints, [[tenderness]], pain on movement and decreased the range of movement is usually found.

==Laboratory findings==
Laboratory tests used to diagnose [[rheumatoid arthritis]] are [[complete blood count]] with differentials, [[erythrocyte sedimentation rate]], [[CRP]], [[Renal function test]], [[LFT]] and [[antibodies]] such as [[rheumatoid factor]], anti-[[CCP]] and anti MCV.

==X-ray==
Hallmark of [[rheumatoid arthritis]] are soft tissue swelling, joint space narrowing and erosions. Hand and wrist findings on xray are subchondral [[cysts]], [[ulnar]] deviation of the [[MCP joint|MCP]] joints, [[boutonniere deformity|boutonniere]] and [[swan neck deformity|swan neck]] deformities, hitchhiker’s thumb deformity, scapholunate dissociation, ulnar translocation and [[ankylosis]]. Feet findings on xray are [[subtalar joint|Subtalar]] joint involvement, posterior [[calcaneus|calcaneal]] tubercle erosion, hammer toe deformity and [[hallux]] [[valgus]]. Findings of shoulder such as distal [[clavicle]] erosions, erosions of the superolateral aspect of the head of the [[humerus]], high riding [[shoulder]] due to subacromial-subdeltoid [[bursitis]]. Knee findigs are [[joint]] effusions, loss of joint space and [[prepatellar bursitis]]. Hip findings are concentric loss of [[joint]] space and acetabular protrusio. Spine findings are [[atlantoaxial]] subluxation, atlantoaxial impaction: cephalad migration of C2,[[osteoporosis]] and [[osteoporotic bones|osteoporotic]] fractures and erosion of spinous processes.

==ECG==
There are no [[ECG]] findings associated with [[rheumatoid arthritis]]. An ECG may be helpful in the diagnosis of pericarditis associated with [[rheumatoid arthritis]]. Findings of pericarditis are diffuse ST-segment elevation and PR segment depression.

==Ultrasound==
[[Ultrasound]] is helpful in the diagnosis of [[rheumatoid arthritis]]. Findings on an ultrasound suggestive of [[rheumatoid arthritis]] are [[tenosynovitis]], [[bursitis]], [[baker's cyst]] and [[inflammation]] of [[synovium]]. Therapeutic use of [[ultrasound]] are guidance of [[corticosteroid]] injection in the joint and fluid aspiration from the joint effusion.

==CT==
CT scan is a useful non-invasive test used to diagnose the complications of [[rheumatoid arthritis]] such as [[subluxation]] of the alanto-axial joint. It is also used in the presurgical assessment of [[neurological]] symptoms.

==MRI==
MRI is helpful in the diagnosis of early and subacute [[rheumatoid arthritis]]. Findings on MRI diagnostic of [[rheumatoid arthritis]] are [[synovial]] hyperemia, [[synovial]] [[hyperplasia]], [[pannus]], subchondral [[cysts]], erosions, [[effusion]] of [[joints]] and bone marrow [[edema]].
==Other imaging studies==
DEXA Scan is helpful in the diagnosis of the complication of [[rheumatoid arthritis]] such as [[osteoporosis]] and [[osteopenia]].
==Other diagnostic studies==
There are no other diagnostic studies associated with [[rheumatoid arthritis]].

==Treatment==

==Medical Therapy==
The mainstay of treatment of [[rheumatoid arthritis]] is pharmacotherapy. Early diagnosis of [[rheumatoid arthritis]] is helpful in treatment. Choice of treatment depends on various factors such as stage of [[disease]], comorbid conditions, stage of therapy and presence of severe prognostic signs. There are two types of therapies- non-pharmacological therapy and pharmacological therapy. There are different drug regimen depending upon the stage of the disease. In active stage, combined therapy include disease-modifying antirheumatic drugs ([[DMARDs]]) are used along with the [[anti-inflammatory drugs]]. Therapy for resistant disease and flares is the addition of oral [[glucocorticoids]]. In case of the flare, add another [[DMARDs]] with [[methotrexate]] or replace with another [[DMARDs]].

==Surgical Therapy==
The mainstay of treatment for [[rheumatoid arthritis]] is medical therapy. Surgery is usually reserved for patients with debilitating disease, failure of medical therapy and radiographic findings of end-stage [[arthritis]]. Various surgical options used for the treatment of [[RA]] are tenosynovectomy, [[arthroscopic]] or open [[synovectomy]], joint fusion, small joint implant [[arthroplasty]], total joint replacement of deformed joint.

==Primary prevention==
Effective measures for the primary prevention of [[rheumatoid arthritis]] are [[patient education]], [[exercise]], [[Physical Therapy|physical]] and [[occupational therapy]] and cognitive behavioral therapies.
==Secondary prevention==
Effective measures for the secondary prevention of [[rheumatoid arthritis]] are [[calcium]] and [[vitamin D]] supplementation to prevent [[osteoporosis]]. To prevent [[cardiovascular]] complications and recurrent attacks of [[RA]], effective methods are [[exercise]], [[smoking]] cessation, and [[dietary control]].

==Reference==
{{Reflist|2}}

[[Category:Aging-associated diseases]]
[[Category:Arthritis]]
[[Category:Autoimmune diseases]]
[[Category:Diseases involving the fasciae]]
[[Category:Rheumatology]]

{{WH}}
{{WS}}

Rheumatoid arthritis overview

2020-05-10T17:37:37Z

Ayesha A. Khan: /* Causes */

__NOTOC__
{{Rheumatoid arthritis}}
{{CMG}} {{AE}} {{MKK}}

==Overview==
[[Rheumatoid arthritis]] is a chronic inflammatory systemic disease which is found commonly in women and starts in mid 30's to 40's. Cause of [[rheumatoid arthritis]] is idiopathic but there are [[Genetics|genetic]] factors such as [[HLA]]-DR4 cluster, [[Environmental Health Perspectives|environmental]] factors like [[smoking]], hormonal factors like [[hyperprolactinemia]], infectious factors like [[mycoplasma]], epstein-Barr virus ([[EBV]]), [[rubella virus]], [[porphyromonas gingivalis]], socioeconomic, [[psychological]], and lifestyle factors such as [[obesity]] play important role in the development of [[rheumatoid arthritis]]. There is no established system for the classification of [[rheumatoid arthritis]]. Depending on the presentation of [[rheumatoid arthritis]], it is classified into typical classic [[rheumatoid arthritis]] and palindromic [[rheumatism]]. Common symptoms of [[rheumatoid arthritis]] are [[joint pain]] of small joints of the hands, wrist, and forefoot, [[joint swelling]], morning [[joint stiffness]] classically lasting for more than an hour, decreased grip strength, generalized [[aching]], and [[fatigue]], [[weight loss]], [[depression]]. Less common symptoms are [[tingling]] and [[numbness]] in the arm and [[weakness]] in the arm. Anti-CCP antibodies testing is the gold standard test for the diagnosis of [[rheumatoid arthritis]].It has the specificity of about 81-100%, with the sensitivity of 39–94%. Hallmark of [[rheumatoid arthritis]] Xray findings are soft tissue swelling, joint space narrowing, and erosions but it involves small joints with multiple deformities. Findings on an ultrasound suggestive of [[rheumatoid arthritis]] are [[tenosynovitis]], [[bursitis]], [[baker's cyst]] and [[inflammation]] of [[synovium]]. Therapeutic use of [[ultrasound]] is the guidance of [[corticosteroid]] injection in the joint and fluid aspiration from the joint effusion. MRI is helpful in the diagnosis of early and subacute [[rheumatoid arthritis]]. Findings on MRI diagnostic of [[rheumatoid arthritis]] are [[synovial]] hyperemia, [[synovial]] [[hyperplasia]], [[pannus]], subchondral [[cysts]], erosions, effusion of [[joints]] and bone marrow [[edema]]. The mainstay of treatment of [[rheumatoid arthritis]] is pharmacotherapy. There are two types of therapies- non-pharmacological therapy and pharmacological therapy. There are different drug regimen depending upon the stage of the disease. In active stage, combined therapy include disease-modifying antirheumatic drugs ([[DMARDs]]) are used along with the [[anti-inflammatory drugs]]. Therapy for resistant disease and flares is the addition of oral [[glucocorticoids]]. In case of the flare, add another [[DMARDs]] with [[methotrexate]] or replace with another [[DMARDs]]. Surgery is usually reserved for patients with debilitating disease, failure of medical therapy and radiographic findings of end-stage [[arthritis]]. Various surgical options used for the treatment of [[RA]] are tenosynovectomy, [[arthroscopic]] or open [[synovectomy]], joint fusion, small joint implant [[arthroplasty]], total joint replacement of deformed joint. Effective measures for the primary prevention of [[rheumatoid arthritis]] are [[patient education]], [[exercise]], [[Physical Therapy|physical]] and [[occupational therapy]] and cognitive behavioral therapies. Effective measures for the secondary prevention of [[rheumatoid arthritis]] are [[calcium]] and [[vitamin D]] supplementation to prevent [[osteoporosis]]. To prevent [[cardiovascular]] complications and recurrent attacks of [[RA]], effective methods are [[exercise]], [[smoking]] cessation, and [[dietary control]].

==Historical Perspective==
[[Rheumatoid arthritis]] signs and symptoms were first noticed by Augustin Jacob Landre-Beauvais, a resident physician at the Saltpetriere asylum in France, in 1800, He named it as Goutte Asthenique Primitive or Primary Asthenic [[Gout]]. The name [[Rheumatoid arthritis]] coined by Archibald Garrod in 1890. In 1970, [[human leukocyte antigen]] (HLA) was first implicated in the pathogenesis of [[rheumatoid arthritis]].

==Classification==
There is no established system for the classification of [[rheumatoid arthritis]]. Depending on the presentation of [[rheumatoid arthritis]], it is classified into typical classic [[rheumatoid arthritis]] and palindromic [[rheumatism]]. The classification criterion for the diagnosis of rheumatoid arthritis according to American College of Rheumatology, uses 4 parameters including [[joint]] involvement, [[serology]], duration of [[symptoms]], and acute phase reactants. If the score is more than 6 according to this criteria, the disease is classified as definite [[rheumatoid arthritis]].

==Pathophysiology==
[[Rheumatoid arthritis]] is mediated by the combination of a predisposing [[genotype]] upon which [[genetic]] factors, [[Environmental Lung Diseases|environmental]] and [[Microorganisms|microorganism]] also contribute resulting in the [[inflammation]] and destruction of the [[synovial membrane]]. All the factors lead to [[citrullination]] or [[post-translational modifications]], the altered [[peptides]] bind to [[MHC]] protein with shared [[epitopes]] which further lead to [[antigen]] presentation to [[T-cells]].
[[T-cells]] further stimulate [[B-cells]] and cytokines which leads to cartilage damage. [[Mutation]] of [[human leukocyte antigen]] (HLA) genes on [[chromosome]] 6 is involved in pathogenesis of [[rheumatoid arthritis]]. Conditions associated with [[RA]] are [[vasculitis]], [[uveitis]], [[scleritis]], peripheral [[ulcerative keratitis]],
[[interstitial fibrosis]], pulmonary [[nodules]], [[bronchiolitis obliterans]], organizing [[pneumonia]], [[venous thromboembolism]], [[pericarditis]], [[myocarditis]], [[congestive heart failure]], [[atrial fibrillation]], [[sjogren's syndrome]], [[felty's syndrome]].

==Causes==
[[Rheumatoid arthritis]] occurs when your [[immune system]] attacks the [[synovium]] — the lining of the membranes that surround your [[joint]]s.The resulting [[inflammation]] thickens the [[synovium]], which can eventually destroy the [[cartilage]] and [[bone]] within the [[joint]].The [[tendon]]s and [[ligament]]s that hold the [[joint]] together weaken and stretch. Gradually, the joint loses its shape and alignment.Cause of [[rheumatoid arthritis]] is idiopathic but [[Genetics|genetic]] like [[HLA]]-DR4 cluster, [[Environmental Health Perspectives|environmental]] like [[smoking]], hormonal like [[hyperprolactinemia]], [[Immunological|immunologic]], infectious factors like [[mycoplasma]], epstein-Barr virus ([[EBV]]), [[rubella virus]], [[porphyromonas gingivalis]], socioeconomic, [[psychological]], and lifestyle factors such as [[obesity]] play important role in the development of [[rheumatoid arthritis]].

==Differentiating Rheumatoid Arthritis from other Diseases==
[[Rheumatoid arthritis]] must be differentiated from [[osteoarthritis]] , [[septic arthritis]], [[reactive arthritis]], [[Behçet's disease|behcet's disease]] and [[psoriatic arthritis]].

==Epidemiology and Demographics==
The incidence of [[rheumatoid arthritis]] is approximately 40 per 100,000 individuals worldwide. The prevalence of [[rheumatoid arthritis]] is approximately 1 percent in Caucasians per 100,000 individuals worldwide.The peak onset of the disease is between the age of 50 and 75 years. Women are three times more commonly affected by [[rheumatoid arthritis]] than men. The mortality rate of [[rheumatoid arthritis]] is approximately 10%.

==Risk Factors==
Common risk factors in the development of [[rheumatoid arthritis]] are the family history of [[RA]], cigarette [[smoking]], race like more prevalent in Native Americans, strongly associated with [[major histocompatibility complex]] (MHC) class II antigen [[human leukocyte antigen|HLA]]-DR4 and patient with [[silica]] and [[asbestos]] exposure.
Less common risk factors are [[obesity]] and high consumption of red meat.

==Screening==
According to the American College of Rheumatology Guideline, screening for [[rheumatoid arthritis]] is not recommended.

==Natural History, Complications and Prognosis==
The symptoms of [[rheumatoid arthritis]] usually develop in the third to fourth decade of life and start with symptoms such as fatigue, small joints pain and morning stiffness which lasts more than 1 hour. Complication of [[rheumatoid arthritis]] involve all the systems. Cardiac complications are pericarditis, congestive heart failure, endocarditis and myocardial infarction. Pulmonary complications are pleurisy, [[alveolitis]], [[pleural effusion]] and [[pulmonary fibrosis]]. Rheumatological complications are [[joint]] deformities, [[felty's syndrome]], [[sjögren's syndrome]], [[osteoporosis]] and atlantoaxial subluxation. Eye complications such as [[scleritis]] and [[keratitis]]. Renal complications are [[chronic renal failure]] from [[amyloidosis]]. Nervous system complications like [[peripheral neuropathy]] and [[mononeuritis multiplex]]. Prognosis is generally good with early diagnosis and treatment, and 10-year disability rate of patients with rheumatoid arthritis is approximately 40%.

==Diagnostic study of choice==
Anti-CCP antibodies testing is the gold standard test for the diagnosis of [[rheumatoid arthritis]].It has the specificity of about 81-100%, with the [[sensitivity]] of 39–94%.

==History and Symptoms==
Patients with [[rheumatoid arthritis]] may have a positive history of the family history of [[RA]], [[history of smoking]] and other [[autoimmune disease]]. Common symptoms of [[rheumatoid arthritis]] are [[joint pain]] of small joints of the hands, wrist, and forefoot, [[joint swelling]], morning [[joint stiffness]] classically lasting for more than an hour, decreased grip strength, generalized [[aching]], and [[fatigue]], [[weight loss]], [[depression]]. Less common symptoms are [[tingling]] and [[numbness]] in the arm and [[weakness]] in the arm.

==Physical Examination==
Patients with rheumatoid arthritis usually appear fatigued. On skin examination, [[Rheumatoid nodule|rheumatoid nodules]], [[erythema nodosum]], [[atrophy]] of the digital skin, [[palmar erythema]] and diffuse thinning of the skin are found. If there is involvement of eyes, [[Scleritis]] and [[Scleromalacia]] are seen. On auscultation of lungs, there are decreased breath sounds on both sides and [[crackles]] may be present. On palpation of the abdomen, [[hepatomegaly]] and [[splenomegaly]] is usually found when it is associated with [[Felty's syndrome]]. On examination of extremities, [[redness]], and swelling of the affected joints, [[tenderness]], pain on movement and decreased the range of movement is usually found.

==Laboratory findings==
Laboratory tests used to diagnose [[rheumatoid arthritis]] are [[complete blood count]] with differentials, [[erythrocyte sedimentation rate]], [[CRP]], [[Renal function test]], [[LFT]] and [[antibodies]] such as [[rheumatoid factor]], anti-[[CCP]] and anti MCV.

==X-ray==
Hallmark of [[rheumatoid arthritis]] are soft tissue swelling, joint space narrowing and erosions. Hand and wrist findings on xray are subchondral [[cysts]], [[ulnar]] deviation of the [[MCP joint|MCP]] joints, [[boutonniere deformity|boutonniere]] and [[swan neck deformity|swan neck]] deformities, hitchhiker’s thumb deformity, scapholunate dissociation, ulnar translocation and [[ankylosis]]. Feet findings on xray are [[subtalar joint|Subtalar]] joint involvement, posterior [[calcaneus|calcaneal]] tubercle erosion, hammer toe deformity and [[hallux]] [[valgus]]. Findings of shoulder such as distal [[clavicle]] erosions, erosions of the superolateral aspect of the head of the [[humerus]], high riding [[shoulder]] due to subacromial-subdeltoid [[bursitis]]. Knee findigs are [[joint]] effusions, loss of joint space and [[prepatellar bursitis]]. Hip findings are concentric loss of [[joint]] space and acetabular protrusio. Spine findings are [[atlantoaxial]] subluxation, atlantoaxial impaction: cephalad migration of C2,[[osteoporosis]] and [[osteoporotic bones|osteoporotic]] fractures and erosion of spinous processes.

==ECG==
There are no [[ECG]] findings associated with [[rheumatoid arthritis]]. An ECG may be helpful in the diagnosis of pericarditis associated with [[rheumatoid arthritis]]. Findings of pericarditis are diffuse ST-segment elevation and PR segment depression.

==Ultrasound==
[[Ultrasound]] is helpful in the diagnosis of [[rheumatoid arthritis]]. Findings on an ultrasound suggestive of [[rheumatoid arthritis]] are [[tenosynovitis]], [[bursitis]], [[baker's cyst]] and [[inflammation]] of [[synovium]]. Therapeutic use of [[ultrasound]] are guidance of [[corticosteroid]] injection in the joint and fluid aspiration from the joint effusion.

==CT==
CT scan is a useful non-invasive test used to diagnose the complications of [[rheumatoid arthritis]] such as [[subluxation]] of the alanto-axial joint. It is also used in the presurgical assessment of [[neurological]] symptoms.

==MRI==
MRI is helpful in the diagnosis of early and subacute [[rheumatoid arthritis]]. Findings on MRI diagnostic of [[rheumatoid arthritis]] are [[synovial]] hyperemia, [[synovial]] [[hyperplasia]], [[pannus]], subchondral [[cysts]], erosions, [[effusion]] of [[joints]] and bone marrow [[edema]].
==Other imaging studies==
DEXA Scan is helpful in the diagnosis of the complication of [[rheumatoid arthritis]] such as [[osteoporosis]] and [[osteopenia]].
==Other diagnostic studies==
There are no other diagnostic studies associated with [[rheumatoid arthritis]].

==Treatment==

==Medical Therapy==
The mainstay of treatment of [[rheumatoid arthritis]] is pharmacotherapy. Early diagnosis of [[rheumatoid arthritis]] is helpful in treatment. Choice of treatment depends on various factors such as stage of [[disease]], comorbid conditions, stage of therapy and presence of severe prognostic signs. There are two types of therapies- non-pharmacological therapy and pharmacological therapy. There are different drug regimen depending upon the stage of the disease. In active stage, combined therapy include disease-modifying antirheumatic drugs ([[DMARDs]]) are used along with the [[anti-inflammatory drugs]]. Therapy for resistant disease and flares is the addition of oral [[glucocorticoids]]. In case of the flare, add another [[DMARDs]] with [[methotrexate]] or replace with another [[DMARDs]].

==Surgical Therapy==
The mainstay of treatment for [[rheumatoid arthritis]] is medical therapy. Surgery is usually reserved for patients with debilitating disease, failure of medical therapy and radiographic findings of end-stage [[arthritis]]. Various surgical options used for the treatment of [[RA]] are tenosynovectomy, [[arthroscopic]] or open [[synovectomy]], joint fusion, small joint implant [[arthroplasty]], total joint replacement of deformed joint.

==Primary prevention==
Effective measures for the primary prevention of [[rheumatoid arthritis]] are [[patient education]], [[exercise]], [[Physical Therapy|physical]] and [[occupational therapy]] and cognitive behavioral therapies.
==Secondary prevention==
Effective measures for the secondary prevention of [[rheumatoid arthritis]] are [[calcium]] and [[vitamin D]] supplementation to prevent [[osteoporosis]]. To prevent [[cardiovascular]] complications and recurrent attacks of [[RA]], effective methods are [[exercise]], [[smoking]] cessation, and [[dietary control]].

==Reference==
{{Reflist|2}}

[[Category:Aging-associated diseases]]
[[Category:Arthritis]]
[[Category:Autoimmune diseases]]
[[Category:Diseases involving the fasciae]]
[[Category:Rheumatology]]

{{WH}}
{{WS}}

Rheumatoid arthritis overview

2020-05-10T17:34:40Z

Ayesha A. Khan: /* Causes */

__NOTOC__
{{Rheumatoid arthritis}}
{{CMG}} {{AE}} {{MKK}}

==Overview==
[[Rheumatoid arthritis]] is a chronic inflammatory systemic disease which is found commonly in women and starts in mid 30's to 40's. Cause of [[rheumatoid arthritis]] is idiopathic but there are [[Genetics|genetic]] factors such as [[HLA]]-DR4 cluster, [[Environmental Health Perspectives|environmental]] factors like [[smoking]], hormonal factors like [[hyperprolactinemia]], infectious factors like [[mycoplasma]], epstein-Barr virus ([[EBV]]), [[rubella virus]], [[porphyromonas gingivalis]], socioeconomic, [[psychological]], and lifestyle factors such as [[obesity]] play important role in the development of [[rheumatoid arthritis]]. There is no established system for the classification of [[rheumatoid arthritis]]. Depending on the presentation of [[rheumatoid arthritis]], it is classified into typical classic [[rheumatoid arthritis]] and palindromic [[rheumatism]]. Common symptoms of [[rheumatoid arthritis]] are [[joint pain]] of small joints of the hands, wrist, and forefoot, [[joint swelling]], morning [[joint stiffness]] classically lasting for more than an hour, decreased grip strength, generalized [[aching]], and [[fatigue]], [[weight loss]], [[depression]]. Less common symptoms are [[tingling]] and [[numbness]] in the arm and [[weakness]] in the arm. Anti-CCP antibodies testing is the gold standard test for the diagnosis of [[rheumatoid arthritis]].It has the specificity of about 81-100%, with the sensitivity of 39–94%. Hallmark of [[rheumatoid arthritis]] Xray findings are soft tissue swelling, joint space narrowing, and erosions but it involves small joints with multiple deformities. Findings on an ultrasound suggestive of [[rheumatoid arthritis]] are [[tenosynovitis]], [[bursitis]], [[baker's cyst]] and [[inflammation]] of [[synovium]]. Therapeutic use of [[ultrasound]] is the guidance of [[corticosteroid]] injection in the joint and fluid aspiration from the joint effusion. MRI is helpful in the diagnosis of early and subacute [[rheumatoid arthritis]]. Findings on MRI diagnostic of [[rheumatoid arthritis]] are [[synovial]] hyperemia, [[synovial]] [[hyperplasia]], [[pannus]], subchondral [[cysts]], erosions, effusion of [[joints]] and bone marrow [[edema]]. The mainstay of treatment of [[rheumatoid arthritis]] is pharmacotherapy. There are two types of therapies- non-pharmacological therapy and pharmacological therapy. There are different drug regimen depending upon the stage of the disease. In active stage, combined therapy include disease-modifying antirheumatic drugs ([[DMARDs]]) are used along with the [[anti-inflammatory drugs]]. Therapy for resistant disease and flares is the addition of oral [[glucocorticoids]]. In case of the flare, add another [[DMARDs]] with [[methotrexate]] or replace with another [[DMARDs]]. Surgery is usually reserved for patients with debilitating disease, failure of medical therapy and radiographic findings of end-stage [[arthritis]]. Various surgical options used for the treatment of [[RA]] are tenosynovectomy, [[arthroscopic]] or open [[synovectomy]], joint fusion, small joint implant [[arthroplasty]], total joint replacement of deformed joint. Effective measures for the primary prevention of [[rheumatoid arthritis]] are [[patient education]], [[exercise]], [[Physical Therapy|physical]] and [[occupational therapy]] and cognitive behavioral therapies. Effective measures for the secondary prevention of [[rheumatoid arthritis]] are [[calcium]] and [[vitamin D]] supplementation to prevent [[osteoporosis]]. To prevent [[cardiovascular]] complications and recurrent attacks of [[RA]], effective methods are [[exercise]], [[smoking]] cessation, and [[dietary control]].

==Historical Perspective==
[[Rheumatoid arthritis]] signs and symptoms were first noticed by Augustin Jacob Landre-Beauvais, a resident physician at the Saltpetriere asylum in France, in 1800, He named it as Goutte Asthenique Primitive or Primary Asthenic [[Gout]]. The name [[Rheumatoid arthritis]] coined by Archibald Garrod in 1890. In 1970, [[human leukocyte antigen]] (HLA) was first implicated in the pathogenesis of [[rheumatoid arthritis]].

==Classification==
There is no established system for the classification of [[rheumatoid arthritis]]. Depending on the presentation of [[rheumatoid arthritis]], it is classified into typical classic [[rheumatoid arthritis]] and palindromic [[rheumatism]]. The classification criterion for the diagnosis of rheumatoid arthritis according to American College of Rheumatology, uses 4 parameters including [[joint]] involvement, [[serology]], duration of [[symptoms]], and acute phase reactants. If the score is more than 6 according to this criteria, the disease is classified as definite [[rheumatoid arthritis]].

==Pathophysiology==
[[Rheumatoid arthritis]] is mediated by the combination of a predisposing [[genotype]] upon which [[genetic]] factors, [[Environmental Lung Diseases|environmental]] and [[Microorganisms|microorganism]] also contribute resulting in the [[inflammation]] and destruction of the [[synovial membrane]]. All the factors lead to [[citrullination]] or [[post-translational modifications]], the altered [[peptides]] bind to [[MHC]] protein with shared [[epitopes]] which further lead to [[antigen]] presentation to [[T-cells]].
[[T-cells]] further stimulate [[B-cells]] and cytokines which leads to cartilage damage. [[Mutation]] of [[human leukocyte antigen]] (HLA) genes on [[chromosome]] 6 is involved in pathogenesis of [[rheumatoid arthritis]]. Conditions associated with [[RA]] are [[vasculitis]], [[uveitis]], [[scleritis]], peripheral [[ulcerative keratitis]],
[[interstitial fibrosis]], pulmonary [[nodules]], [[bronchiolitis obliterans]], organizing [[pneumonia]], [[venous thromboembolism]], [[pericarditis]], [[myocarditis]], [[congestive heart failure]], [[atrial fibrillation]], [[sjogren's syndrome]], [[felty's syndrome]].

==Causes==
Rheumatoid arthritis occurs when your immune system attacks the synovium — the lining of the membranes that surround your joints.The resulting inflammation thickens the synovium, which can eventually destroy the cartilage and bone within the joint.The tendons and ligaments that hold the joint together weaken and stretch. Gradually, the joint loses its shape and alignment.Cause of [[rheumatoid arthritis]] is idiopathic but [[Genetics|genetic]] like [[HLA]]-DR4 cluster, [[Environmental Health Perspectives|environmental]] like [[smoking]], hormonal like [[hyperprolactinemia]], [[Immunological|immunologic]], infectious factors like [[mycoplasma]], epstein-Barr virus ([[EBV]]), [[rubella virus]], [[porphyromonas gingivalis]], socioeconomic, [[psychological]], and lifestyle factors such as [[obesity]] play important role in the development of [[rheumatoid arthritis]].

==Differentiating Rheumatoid Arthritis from other Diseases==
[[Rheumatoid arthritis]] must be differentiated from [[osteoarthritis]] , [[septic arthritis]], [[reactive arthritis]], [[Behçet's disease|behcet's disease]] and [[psoriatic arthritis]].

==Epidemiology and Demographics==
The incidence of [[rheumatoid arthritis]] is approximately 40 per 100,000 individuals worldwide. The prevalence of [[rheumatoid arthritis]] is approximately 1 percent in Caucasians per 100,000 individuals worldwide.The peak onset of the disease is between the age of 50 and 75 years. Women are three times more commonly affected by [[rheumatoid arthritis]] than men. The mortality rate of [[rheumatoid arthritis]] is approximately 10%.

==Risk Factors==
Common risk factors in the development of [[rheumatoid arthritis]] are the family history of [[RA]], cigarette [[smoking]], race like more prevalent in Native Americans, strongly associated with [[major histocompatibility complex]] (MHC) class II antigen [[human leukocyte antigen|HLA]]-DR4 and patient with [[silica]] and [[asbestos]] exposure.
Less common risk factors are [[obesity]] and high consumption of red meat.

==Screening==
According to the American College of Rheumatology Guideline, screening for [[rheumatoid arthritis]] is not recommended.

==Natural History, Complications and Prognosis==
The symptoms of [[rheumatoid arthritis]] usually develop in the third to fourth decade of life and start with symptoms such as fatigue, small joints pain and morning stiffness which lasts more than 1 hour. Complication of [[rheumatoid arthritis]] involve all the systems. Cardiac complications are pericarditis, congestive heart failure, endocarditis and myocardial infarction. Pulmonary complications are pleurisy, [[alveolitis]], [[pleural effusion]] and [[pulmonary fibrosis]]. Rheumatological complications are [[joint]] deformities, [[felty's syndrome]], [[sjögren's syndrome]], [[osteoporosis]] and atlantoaxial subluxation. Eye complications such as [[scleritis]] and [[keratitis]]. Renal complications are [[chronic renal failure]] from [[amyloidosis]]. Nervous system complications like [[peripheral neuropathy]] and [[mononeuritis multiplex]]. Prognosis is generally good with early diagnosis and treatment, and 10-year disability rate of patients with rheumatoid arthritis is approximately 40%.

==Diagnostic study of choice==
Anti-CCP antibodies testing is the gold standard test for the diagnosis of [[rheumatoid arthritis]].It has the specificity of about 81-100%, with the [[sensitivity]] of 39–94%.

==History and Symptoms==
Patients with [[rheumatoid arthritis]] may have a positive history of the family history of [[RA]], [[history of smoking]] and other [[autoimmune disease]]. Common symptoms of [[rheumatoid arthritis]] are [[joint pain]] of small joints of the hands, wrist, and forefoot, [[joint swelling]], morning [[joint stiffness]] classically lasting for more than an hour, decreased grip strength, generalized [[aching]], and [[fatigue]], [[weight loss]], [[depression]]. Less common symptoms are [[tingling]] and [[numbness]] in the arm and [[weakness]] in the arm.

==Physical Examination==
Patients with rheumatoid arthritis usually appear fatigued. On skin examination, [[Rheumatoid nodule|rheumatoid nodules]], [[erythema nodosum]], [[atrophy]] of the digital skin, [[palmar erythema]] and diffuse thinning of the skin are found. If there is involvement of eyes, [[Scleritis]] and [[Scleromalacia]] are seen. On auscultation of lungs, there are decreased breath sounds on both sides and [[crackles]] may be present. On palpation of the abdomen, [[hepatomegaly]] and [[splenomegaly]] is usually found when it is associated with [[Felty's syndrome]]. On examination of extremities, [[redness]], and swelling of the affected joints, [[tenderness]], pain on movement and decreased the range of movement is usually found.

==Laboratory findings==
Laboratory tests used to diagnose [[rheumatoid arthritis]] are [[complete blood count]] with differentials, [[erythrocyte sedimentation rate]], [[CRP]], [[Renal function test]], [[LFT]] and [[antibodies]] such as [[rheumatoid factor]], anti-[[CCP]] and anti MCV.

==X-ray==
Hallmark of [[rheumatoid arthritis]] are soft tissue swelling, joint space narrowing and erosions. Hand and wrist findings on xray are subchondral [[cysts]], [[ulnar]] deviation of the [[MCP joint|MCP]] joints, [[boutonniere deformity|boutonniere]] and [[swan neck deformity|swan neck]] deformities, hitchhiker’s thumb deformity, scapholunate dissociation, ulnar translocation and [[ankylosis]]. Feet findings on xray are [[subtalar joint|Subtalar]] joint involvement, posterior [[calcaneus|calcaneal]] tubercle erosion, hammer toe deformity and [[hallux]] [[valgus]]. Findings of shoulder such as distal [[clavicle]] erosions, erosions of the superolateral aspect of the head of the [[humerus]], high riding [[shoulder]] due to subacromial-subdeltoid [[bursitis]]. Knee findigs are [[joint]] effusions, loss of joint space and [[prepatellar bursitis]]. Hip findings are concentric loss of [[joint]] space and acetabular protrusio. Spine findings are [[atlantoaxial]] subluxation, atlantoaxial impaction: cephalad migration of C2,[[osteoporosis]] and [[osteoporotic bones|osteoporotic]] fractures and erosion of spinous processes.

==ECG==
There are no [[ECG]] findings associated with [[rheumatoid arthritis]]. An ECG may be helpful in the diagnosis of pericarditis associated with [[rheumatoid arthritis]]. Findings of pericarditis are diffuse ST-segment elevation and PR segment depression.

==Ultrasound==
[[Ultrasound]] is helpful in the diagnosis of [[rheumatoid arthritis]]. Findings on an ultrasound suggestive of [[rheumatoid arthritis]] are [[tenosynovitis]], [[bursitis]], [[baker's cyst]] and [[inflammation]] of [[synovium]]. Therapeutic use of [[ultrasound]] are guidance of [[corticosteroid]] injection in the joint and fluid aspiration from the joint effusion.

==CT==
CT scan is a useful non-invasive test used to diagnose the complications of [[rheumatoid arthritis]] such as [[subluxation]] of the alanto-axial joint. It is also used in the presurgical assessment of [[neurological]] symptoms.

==MRI==
MRI is helpful in the diagnosis of early and subacute [[rheumatoid arthritis]]. Findings on MRI diagnostic of [[rheumatoid arthritis]] are [[synovial]] hyperemia, [[synovial]] [[hyperplasia]], [[pannus]], subchondral [[cysts]], erosions, [[effusion]] of [[joints]] and bone marrow [[edema]].
==Other imaging studies==
DEXA Scan is helpful in the diagnosis of the complication of [[rheumatoid arthritis]] such as [[osteoporosis]] and [[osteopenia]].
==Other diagnostic studies==
There are no other diagnostic studies associated with [[rheumatoid arthritis]].

==Treatment==

==Medical Therapy==
The mainstay of treatment of [[rheumatoid arthritis]] is pharmacotherapy. Early diagnosis of [[rheumatoid arthritis]] is helpful in treatment. Choice of treatment depends on various factors such as stage of [[disease]], comorbid conditions, stage of therapy and presence of severe prognostic signs. There are two types of therapies- non-pharmacological therapy and pharmacological therapy. There are different drug regimen depending upon the stage of the disease. In active stage, combined therapy include disease-modifying antirheumatic drugs ([[DMARDs]]) are used along with the [[anti-inflammatory drugs]]. Therapy for resistant disease and flares is the addition of oral [[glucocorticoids]]. In case of the flare, add another [[DMARDs]] with [[methotrexate]] or replace with another [[DMARDs]].

==Surgical Therapy==
The mainstay of treatment for [[rheumatoid arthritis]] is medical therapy. Surgery is usually reserved for patients with debilitating disease, failure of medical therapy and radiographic findings of end-stage [[arthritis]]. Various surgical options used for the treatment of [[RA]] are tenosynovectomy, [[arthroscopic]] or open [[synovectomy]], joint fusion, small joint implant [[arthroplasty]], total joint replacement of deformed joint.

==Primary prevention==
Effective measures for the primary prevention of [[rheumatoid arthritis]] are [[patient education]], [[exercise]], [[Physical Therapy|physical]] and [[occupational therapy]] and cognitive behavioral therapies.
==Secondary prevention==
Effective measures for the secondary prevention of [[rheumatoid arthritis]] are [[calcium]] and [[vitamin D]] supplementation to prevent [[osteoporosis]]. To prevent [[cardiovascular]] complications and recurrent attacks of [[RA]], effective methods are [[exercise]], [[smoking]] cessation, and [[dietary control]].

==Reference==
{{Reflist|2}}

[[Category:Aging-associated diseases]]
[[Category:Arthritis]]
[[Category:Autoimmune diseases]]
[[Category:Diseases involving the fasciae]]
[[Category:Rheumatology]]

{{WH}}
{{WS}}

Rheumatology

2020-05-09T15:40:35Z

Ayesha A. Khan: /* Rheumatism */

{{SI}}

{{CMG}} {{AE}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]]

==Overview==
'''[[Rheumatology]]''', a subspecialty of [[internal medicine]] and [[pediatrics]] is devoted to the [[diagnosis]] and therapy of '''[[rheumatic]] diseases'''. The term originates from the Greek ''rheuma'', meaning "that which flows as a river or stream" and the suffix ''-ology'', meaning "the study of". Rheumatologists mainly deal with problems involving the joints and the allied conditions of connective tissue.

[[Rheumatology]] is a rapidly evolving specialty; new scientific discoveries related to this specialty are largely related to better understanding of [[immunology]] of these disorders. [[Pathogenesis]] of major rheumatological disorders is now described as auto immune disorders. [[Immunology]] explains [[pathogenesis]] and the characteristics of [[rheumatological]] disorders, and the new treatment modalities are also based on [[immunology]], better understanding of [[genetic]] basis of rheumatological disorders makes [[rheumatology]] a specialty rapidly developing as a specialty based on new scientific discoveries. New treatment modalities are based on scientific research on [[immunology]], [[cytokines]], [[T lymphocytes]], [[B lymphocytes]] and the future therapies may be directed more towards [[gene]] therapy as well. Currently, practice of [[rheumatology]] is largely based on clinical research, evidence based medical treatment of [[rheumatological]] disorders has helped patients with [[rheumatism]] lead a near normal life without any disabilities. Those clinicians specialized on this [[specialty]] are called [[rheumatologists]].

== Rheumatism ==

'''[[Rheumatism]]''' is a non-specific term used to describe any painful disorder affecting the loco-motor system including [[joints]], [[muscles]], [[connective tissues]], [[soft tissues]] around the [[joints]] and [[bones]]. The term rheumatism is also used to describe rheumatic fever affecting [[heart valves]]. However, the medical profession use specific terms to describe rheumatological disorders such as [[rheumatoid arthritis]], [[ankylosing spondylitis]], [[gout]] and [[systemic lupus erythematosus]] and so on in the medical literature.

[[Rheumatology]] is now emerging as an important clinical specialty all over the world, along with well organized post graduate training programs organized for the postgraduate trainees in this field. The term describing clinicians as "[[rheumatologists]]" is now a well established term in the medical community, even though it is not well described in language dictionaries. Rheumatologists all over the world are now capable of treating most of the chronic rheumatological disorders with a much better outcome for the patients that is with the discovery of new disease modifying agents called biologics which is now a well established form of [[treatment]] for the patients suffering with chronic and disabling joint disorders. Large proportion of patients with [[rheumatoid arthritis]], up to seventy percent according to some studies can now be cured with the introduction and wide spread use of biologic treatment for the treatment of arthritic disorders since the beginning of twenty first century.

== Rheumatologist ==
'''Rheumatologist.''' (Consultant Rheumatologist.)

Rheumatologist is a clinician specialized in the field of medical subspecialty called rheumatology and hold either a Doctor of Osteopathy degree (D.O.) or Doctor of Medicine Degree (M.D.). Training in this field requires four years undergraduate school, 4 years of medical school, and then postgraduate training. Rheumatologists are internists, physicians or pediatricians who are qualified by additional postgraduate training and experience in the diagnosis and treatment of arthritis and other diseases of the joints, muscles and bones. Many rheumatologists also conduct research to determine the cause and better treatments for these disabling and sometimes fatal diseases. Treatment modalities are also based on scientific research, currently, practice of rheumatology is largely evidence based. Those clinicians specialized on this specialty are called rheumatologists.

Rheumatologists treat arthritis, certain autoimmune diseases, musculoskeletal pain disorders and osteoporosis. There are more than 200 types of these diseases, including rheumatoid arthritis, osteoarthritis, gout, lupus, back pain, osteoporosis, fibromyalgia and [[tendinitis]]. Some of these are very serious diseases that can be difficult to diagnose and treat. They treat soft tissue problems related to musculoskeletal system sports related soft tissue disorders and the specialty is also interrelated with physiotherapy, physical medicine and rehabilitation of disabled patients. Patient education programmes and occupational therapy is also goes hand in hand with this specialty.

There are many '''international organizations representing Rheumatologists''' all over the world. American College of Rheumatology( ACR), the European League Against Rheumatism (EULAR), Asia Pacific League of Associations for Rheumatology(APLAR), International League of Associations for Rheumatology (ILAR) are the main international organizations established and organizing many activities related to this specialty, these organizations strive to propagate and consolidate Rheumatology endeavors internationally , furthermore, there are Associations and Colleges of Rheumatology representing Rheumatologists from each and every nation scattered throughout the world which represent the above mentioned organizations from each nation. Rheumatologists are physicians specialized in rheumatic diseases.
For example, there are approximately 480 consultant rheumatologists in the UK. Rheumatologists are increasing in numbers in all countries, as there is an increasing demand for specialists on this field with an increasing population of ageing patients who need specialized treatment.

== Diseases ==
Diseases diagnosed or managed by the rheumatologist include:

*[[Rheumatoid arthritis]]
*[[lupus erythematosus]]
*[[Sjögren's syndrome]]
*[[scleroderma]] (systemic sclerosis)
*[[dermatomyositis]]
*[[polychondritis]]
*[[polymyositis]]
*[[polymyalgia rheumatica]]
*[[osteoarthritis]]
*[[septic arthritis]]
*[[fibromyalgia]]
*[[sarcoidosis]]
*[[gout]], [[pseudogout]]
*[[spondyloarthropathy|spondyloarthropathies]]
**[[ankylosing spondylitis]]
**[[reactive arthritis]]
**[[psoriatic arthropathy]]
**[[enteropathic spondylitis]]
**[[reactive arthropathy]]
*[[vasculitis]]
**[[polyarteritis nodosa]]
**[[Henoch-Schönlein purpura]]
**[[serum sickness]]
**[[Wegener's granulomatosis]]
**[[giant cell arteritis]]
**[[temporal arteritis]]
**[[Takayasu's arteritis]]
**[[Behçet's syndrome]]
**[[Kawasaki's disease]] (mucocutaneous lymph node syndrome)
**[[Buerger's disease]] ([[thromboangiitis obliterans]])
*Juvenile Idiopathic Arthritis (JIA)



== Diagnosis ==
Apart from an extensive medical history, there are useful methods of diagnosis both performed easy enough in a [[physical examination]] and, on the other hand, more complicated ones, often requiring a rheumatologist or other specialised physicians.

===Physical examination===
Following are examples of methods of diagnosis able to be performed in a normal physical examination.
* [[Schober's test]] tests the flexion of the [[lower back]].

===Specialised===
* [[Medical laboratory|Laboratory]] [[blood test|tests]] (e.g. [[erythrocyte sedimentation rate]], [[rheumatoid factor]])
* [[X-ray]]s of affected joints and other imaging methods
* [[Cytology]] and [[clinical chemistry|chemical pathology]] of fluid aspirated from affected joints (e.g. to differentiate between [[septic arthritis]] and [[gout]])

== Treatment ==
Most rheumatic diseases are treated with [[analgesic]]s, [[NSAID]]s (Non-Steroid Anti-Inflammatory Drugs), [[steroid]]s (in serious cases), [[DMARD]]s (Disease-Modifying Anti-Rheumatic Drugs), [[monoclonal antibody|monoclonal antibodies]], such as [[infliximab]] and [[adalimumab]], and the soluble TNF receptor [[etanercept]].

[[Physiotherapy]] is vital in the treatment of many rheumatological disorders. [[Occupational therapy]] can help patients finding alternative ways for common movements which would otherwise be restricted by their disease.

== Scientific research ==
Recently, a large body of scientific research deals with the background of [[autoimmune disease]], the cause of many rheumatic disorders. Also, the field of [[osteoimmunology]] has emerged to further examine the interactions between the immune system, joints and bones. Epidemiological studies and medication trials are also being conducted.

==External links==
*[http://www.institutferran.org IFR: Institut Ferran de Reumatologia].
*[http://www.aplar.org/index.html APLAR]

{{Medicine}}

[[Category:Rheumatology|*]]
[[Category:Subjects taught in medical school]]

[[de:Rheumatologie]]
[[et:Reumatoloogia]]
[[es:Reumatología]]
[[eu:Erreumatologia]]
[[fr:Rhumatologie]]
[[io:Reumatologio]]
[[id:Rematologi]]
[[it:Reumatologia]]
[[he:ראומטולוגיה]]
[[ja:リウマチ学]]
[[no:Revmatologi]]
[[pl:Reumatologia]]
[[pt:Reumatologia]]
[[ru:Ревматология]]
[[sv:Reumatologi]]
[[tr:Romatoloji]]

{{WH}}
{{WS}}

Rheumatology

2020-05-09T15:36:57Z

Ayesha A. Khan:

{{SI}}

{{CMG}} {{AE}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]]

==Overview==
'''[[Rheumatology]]''', a subspecialty of [[internal medicine]] and [[pediatrics]] is devoted to the [[diagnosis]] and therapy of '''[[rheumatic]] diseases'''. The term originates from the Greek ''rheuma'', meaning "that which flows as a river or stream" and the suffix ''-ology'', meaning "the study of". Rheumatologists mainly deal with problems involving the joints and the allied conditions of connective tissue.

[[Rheumatology]] is a rapidly evolving specialty; new scientific discoveries related to this specialty are largely related to better understanding of [[immunology]] of these disorders. [[Pathogenesis]] of major rheumatological disorders is now described as auto immune disorders. [[Immunology]] explains [[pathogenesis]] and the characteristics of [[rheumatological]] disorders, and the new treatment modalities are also based on [[immunology]], better understanding of [[genetic]] basis of rheumatological disorders makes [[rheumatology]] a specialty rapidly developing as a specialty based on new scientific discoveries. New treatment modalities are based on scientific research on [[immunology]], [[cytokines]], [[T lymphocytes]], [[B lymphocytes]] and the future therapies may be directed more towards [[gene]] therapy as well. Currently, practice of [[rheumatology]] is largely based on clinical research, evidence based medical treatment of [[rheumatological]] disorders has helped patients with [[rheumatism]] lead a near normal life without any disabilities. Those clinicians specialized on this [[specialty]] are called [[rheumatologists]].

== Rheumatism ==

'''Rheumatism''' is a non-specific term used to describe any painful disorder affecting the loco-motor system including joints, muscles, connective tissues, soft tissues around the joints and bones. The term rheumatism is also used to describe rheumatic fever affecting heart valves. However, the medical profession use specific terms to describe rheumatological disorders such as rheumatoid arthritis, ankylosing spondylitis, gout and systemic lupus erythematosus and so on in the medical literature.

Rheumatology is now emerging as an important clinical specialty all over the world, along with well organized post graduate training programs organized for the postgraduate trainees in this field. The term describing clinicians as "rheumatologists" is now a well established term in the medical community, even though it is not well described in language dictionaries. Rheumatologists all over the world are now capable of treating most of the chronic rheumatological disorders with a much better outcome for the patients that is with the discovery of new disease modifying agents called biologics which is now a well established form of treatment for the patients suffering with chronic and disabling joint disorders. Large proportion of patients with rheumatoid arthritis, up to seventy percent according to some studies can now be cured with the introduction and wide spread use of biologic treatment for the treatment of arthritic disorders since the beginning of twenty first century.

== Rheumatologist ==
'''Rheumatologist.''' (Consultant Rheumatologist.)

Rheumatologist is a clinician specialized in the field of medical subspecialty called rheumatology and hold either a Doctor of Osteopathy degree (D.O.) or Doctor of Medicine Degree (M.D.). Training in this field requires four years undergraduate school, 4 years of medical school, and then postgraduate training. Rheumatologists are internists, physicians or pediatricians who are qualified by additional postgraduate training and experience in the diagnosis and treatment of arthritis and other diseases of the joints, muscles and bones. Many rheumatologists also conduct research to determine the cause and better treatments for these disabling and sometimes fatal diseases. Treatment modalities are also based on scientific research, currently, practice of rheumatology is largely evidence based. Those clinicians specialized on this specialty are called rheumatologists.

Rheumatologists treat arthritis, certain autoimmune diseases, musculoskeletal pain disorders and osteoporosis. There are more than 200 types of these diseases, including rheumatoid arthritis, osteoarthritis, gout, lupus, back pain, osteoporosis, fibromyalgia and [[tendinitis]]. Some of these are very serious diseases that can be difficult to diagnose and treat. They treat soft tissue problems related to musculoskeletal system sports related soft tissue disorders and the specialty is also interrelated with physiotherapy, physical medicine and rehabilitation of disabled patients. Patient education programmes and occupational therapy is also goes hand in hand with this specialty.

There are many '''international organizations representing Rheumatologists''' all over the world. American College of Rheumatology( ACR), the European League Against Rheumatism (EULAR), Asia Pacific League of Associations for Rheumatology(APLAR), International League of Associations for Rheumatology (ILAR) are the main international organizations established and organizing many activities related to this specialty, these organizations strive to propagate and consolidate Rheumatology endeavors internationally , furthermore, there are Associations and Colleges of Rheumatology representing Rheumatologists from each and every nation scattered throughout the world which represent the above mentioned organizations from each nation. Rheumatologists are physicians specialized in rheumatic diseases.
For example, there are approximately 480 consultant rheumatologists in the UK. Rheumatologists are increasing in numbers in all countries, as there is an increasing demand for specialists on this field with an increasing population of ageing patients who need specialized treatment.

== Diseases ==
Diseases diagnosed or managed by the rheumatologist include:

*[[Rheumatoid arthritis]]
*[[lupus erythematosus]]
*[[Sjögren's syndrome]]
*[[scleroderma]] (systemic sclerosis)
*[[dermatomyositis]]
*[[polychondritis]]
*[[polymyositis]]
*[[polymyalgia rheumatica]]
*[[osteoarthritis]]
*[[septic arthritis]]
*[[fibromyalgia]]
*[[sarcoidosis]]
*[[gout]], [[pseudogout]]
*[[spondyloarthropathy|spondyloarthropathies]]
**[[ankylosing spondylitis]]
**[[reactive arthritis]]
**[[psoriatic arthropathy]]
**[[enteropathic spondylitis]]
**[[reactive arthropathy]]
*[[vasculitis]]
**[[polyarteritis nodosa]]
**[[Henoch-Schönlein purpura]]
**[[serum sickness]]
**[[Wegener's granulomatosis]]
**[[giant cell arteritis]]
**[[temporal arteritis]]
**[[Takayasu's arteritis]]
**[[Behçet's syndrome]]
**[[Kawasaki's disease]] (mucocutaneous lymph node syndrome)
**[[Buerger's disease]] ([[thromboangiitis obliterans]])
*Juvenile Idiopathic Arthritis (JIA)



== Diagnosis ==
Apart from an extensive medical history, there are useful methods of diagnosis both performed easy enough in a [[physical examination]] and, on the other hand, more complicated ones, often requiring a rheumatologist or other specialised physicians.

===Physical examination===
Following are examples of methods of diagnosis able to be performed in a normal physical examination.
* [[Schober's test]] tests the flexion of the [[lower back]].

===Specialised===
* [[Medical laboratory|Laboratory]] [[blood test|tests]] (e.g. [[erythrocyte sedimentation rate]], [[rheumatoid factor]])
* [[X-ray]]s of affected joints and other imaging methods
* [[Cytology]] and [[clinical chemistry|chemical pathology]] of fluid aspirated from affected joints (e.g. to differentiate between [[septic arthritis]] and [[gout]])

== Treatment ==
Most rheumatic diseases are treated with [[analgesic]]s, [[NSAID]]s (Non-Steroid Anti-Inflammatory Drugs), [[steroid]]s (in serious cases), [[DMARD]]s (Disease-Modifying Anti-Rheumatic Drugs), [[monoclonal antibody|monoclonal antibodies]], such as [[infliximab]] and [[adalimumab]], and the soluble TNF receptor [[etanercept]].

[[Physiotherapy]] is vital in the treatment of many rheumatological disorders. [[Occupational therapy]] can help patients finding alternative ways for common movements which would otherwise be restricted by their disease.

== Scientific research ==
Recently, a large body of scientific research deals with the background of [[autoimmune disease]], the cause of many rheumatic disorders. Also, the field of [[osteoimmunology]] has emerged to further examine the interactions between the immune system, joints and bones. Epidemiological studies and medication trials are also being conducted.

==External links==
*[http://www.institutferran.org IFR: Institut Ferran de Reumatologia].
*[http://www.aplar.org/index.html APLAR]

{{Medicine}}

[[Category:Rheumatology|*]]
[[Category:Subjects taught in medical school]]

[[de:Rheumatologie]]
[[et:Reumatoloogia]]
[[es:Reumatología]]
[[eu:Erreumatologia]]
[[fr:Rhumatologie]]
[[io:Reumatologio]]
[[id:Rematologi]]
[[it:Reumatologia]]
[[he:ראומטולוגיה]]
[[ja:リウマチ学]]
[[no:Revmatologi]]
[[pl:Reumatologia]]
[[pt:Reumatologia]]
[[ru:Ревматология]]
[[sv:Reumatologi]]
[[tr:Romatoloji]]

{{WH}}
{{WS}}

Rheumatology

2020-05-09T15:35:11Z

Ayesha A. Khan:

{{SI}}

{{CMG}}

==Overview==
'''[[Rheumatology]]''', a subspecialty of [[internal medicine]] and [[pediatrics]] is devoted to the [[diagnosis]] and therapy of '''[[rheumatic]] diseases'''. The term originates from the Greek ''rheuma'', meaning "that which flows as a river or stream" and the suffix ''-ology'', meaning "the study of". Rheumatologists mainly deal with problems involving the joints and the allied conditions of connective tissue.

[[Rheumatology]] is a rapidly evolving specialty; new scientific discoveries related to this specialty are largely related to better understanding of [[immunology]] of these disorders. [[Pathogenesis]] of major rheumatological disorders is now described as auto immune disorders. [[Immunology]] explains [[pathogenesis]] and the characteristics of [[rheumatological]] disorders, and the new treatment modalities are also based on [[immunology]], better understanding of [[genetic]] basis of rheumatological disorders makes [[rheumatology]] a specialty rapidly developing as a specialty based on new scientific discoveries. New treatment modalities are based on scientific research on [[immunology]], [[cytokines]], [[T lymphocytes]], [[B lymphocytes]] and the future therapies may be directed more towards [[gene]] therapy as well. Currently, practice of [[rheumatology]] is largely based on clinical research, evidence based medical treatment of [[rheumatological]] disorders has helped patients with [[rheumatism]] lead a near normal life without any disabilities. Those clinicians specialized on this [[specialty]] are called [[rheumatologists]].

== Rheumatism ==

'''Rheumatism''' is a non-specific term used to describe any painful disorder affecting the loco-motor system including joints, muscles, connective tissues, soft tissues around the joints and bones. The term rheumatism is also used to describe rheumatic fever affecting heart valves. However, the medical profession use specific terms to describe rheumatological disorders such as rheumatoid arthritis, ankylosing spondylitis, gout and systemic lupus erythematosus and so on in the medical literature.

Rheumatology is now emerging as an important clinical specialty all over the world, along with well organized post graduate training programs organized for the postgraduate trainees in this field. The term describing clinicians as "rheumatologists" is now a well established term in the medical community, even though it is not well described in language dictionaries. Rheumatologists all over the world are now capable of treating most of the chronic rheumatological disorders with a much better outcome for the patients that is with the discovery of new disease modifying agents called biologics which is now a well established form of treatment for the patients suffering with chronic and disabling joint disorders. Large proportion of patients with rheumatoid arthritis, up to seventy percent according to some studies can now be cured with the introduction and wide spread use of biologic treatment for the treatment of arthritic disorders since the beginning of twenty first century.

== Rheumatologist ==
'''Rheumatologist.''' (Consultant Rheumatologist.)

Rheumatologist is a clinician specialized in the field of medical subspecialty called rheumatology and hold either a Doctor of Osteopathy degree (D.O.) or Doctor of Medicine Degree (M.D.). Training in this field requires four years undergraduate school, 4 years of medical school, and then postgraduate training. Rheumatologists are internists, physicians or pediatricians who are qualified by additional postgraduate training and experience in the diagnosis and treatment of arthritis and other diseases of the joints, muscles and bones. Many rheumatologists also conduct research to determine the cause and better treatments for these disabling and sometimes fatal diseases. Treatment modalities are also based on scientific research, currently, practice of rheumatology is largely evidence based. Those clinicians specialized on this specialty are called rheumatologists.

Rheumatologists treat arthritis, certain autoimmune diseases, musculoskeletal pain disorders and osteoporosis. There are more than 200 types of these diseases, including rheumatoid arthritis, osteoarthritis, gout, lupus, back pain, osteoporosis, fibromyalgia and [[tendinitis]]. Some of these are very serious diseases that can be difficult to diagnose and treat. They treat soft tissue problems related to musculoskeletal system sports related soft tissue disorders and the specialty is also interrelated with physiotherapy, physical medicine and rehabilitation of disabled patients. Patient education programmes and occupational therapy is also goes hand in hand with this specialty.

There are many '''international organizations representing Rheumatologists''' all over the world. American College of Rheumatology( ACR), the European League Against Rheumatism (EULAR), Asia Pacific League of Associations for Rheumatology(APLAR), International League of Associations for Rheumatology (ILAR) are the main international organizations established and organizing many activities related to this specialty, these organizations strive to propagate and consolidate Rheumatology endeavors internationally , furthermore, there are Associations and Colleges of Rheumatology representing Rheumatologists from each and every nation scattered throughout the world which represent the above mentioned organizations from each nation. Rheumatologists are physicians specialized in rheumatic diseases.
For example, there are approximately 480 consultant rheumatologists in the UK. Rheumatologists are increasing in numbers in all countries, as there is an increasing demand for specialists on this field with an increasing population of ageing patients who need specialized treatment.

== Diseases ==
Diseases diagnosed or managed by the rheumatologist include:

*[[Rheumatoid arthritis]]
*[[lupus erythematosus]]
*[[Sjögren's syndrome]]
*[[scleroderma]] (systemic sclerosis)
*[[dermatomyositis]]
*[[polychondritis]]
*[[polymyositis]]
*[[polymyalgia rheumatica]]
*[[osteoarthritis]]
*[[septic arthritis]]
*[[fibromyalgia]]
*[[sarcoidosis]]
*[[gout]], [[pseudogout]]
*[[spondyloarthropathy|spondyloarthropathies]]
**[[ankylosing spondylitis]]
**[[reactive arthritis]]
**[[psoriatic arthropathy]]
**[[enteropathic spondylitis]]
**[[reactive arthropathy]]
*[[vasculitis]]
**[[polyarteritis nodosa]]
**[[Henoch-Schönlein purpura]]
**[[serum sickness]]
**[[Wegener's granulomatosis]]
**[[giant cell arteritis]]
**[[temporal arteritis]]
**[[Takayasu's arteritis]]
**[[Behçet's syndrome]]
**[[Kawasaki's disease]] (mucocutaneous lymph node syndrome)
**[[Buerger's disease]] ([[thromboangiitis obliterans]])
*Juvenile Idiopathic Arthritis (JIA)



== Diagnosis ==
Apart from an extensive medical history, there are useful methods of diagnosis both performed easy enough in a [[physical examination]] and, on the other hand, more complicated ones, often requiring a rheumatologist or other specialised physicians.

===Physical examination===
Following are examples of methods of diagnosis able to be performed in a normal physical examination.
* [[Schober's test]] tests the flexion of the [[lower back]].

===Specialised===
* [[Medical laboratory|Laboratory]] [[blood test|tests]] (e.g. [[erythrocyte sedimentation rate]], [[rheumatoid factor]])
* [[X-ray]]s of affected joints and other imaging methods
* [[Cytology]] and [[clinical chemistry|chemical pathology]] of fluid aspirated from affected joints (e.g. to differentiate between [[septic arthritis]] and [[gout]])

== Treatment ==
Most rheumatic diseases are treated with [[analgesic]]s, [[NSAID]]s (Non-Steroid Anti-Inflammatory Drugs), [[steroid]]s (in serious cases), [[DMARD]]s (Disease-Modifying Anti-Rheumatic Drugs), [[monoclonal antibody|monoclonal antibodies]], such as [[infliximab]] and [[adalimumab]], and the soluble TNF receptor [[etanercept]].

[[Physiotherapy]] is vital in the treatment of many rheumatological disorders. [[Occupational therapy]] can help patients finding alternative ways for common movements which would otherwise be restricted by their disease.

== Scientific research ==
Recently, a large body of scientific research deals with the background of [[autoimmune disease]], the cause of many rheumatic disorders. Also, the field of [[osteoimmunology]] has emerged to further examine the interactions between the immune system, joints and bones. Epidemiological studies and medication trials are also being conducted.

==External links==
*[http://www.institutferran.org IFR: Institut Ferran de Reumatologia].
*[http://www.aplar.org/index.html APLAR]

{{Medicine}}

[[Category:Rheumatology|*]]
[[Category:Subjects taught in medical school]]

[[de:Rheumatologie]]
[[et:Reumatoloogia]]
[[es:Reumatología]]
[[eu:Erreumatologia]]
[[fr:Rhumatologie]]
[[io:Reumatologio]]
[[id:Rematologi]]
[[it:Reumatologia]]
[[he:ראומטולוגיה]]
[[ja:リウマチ学]]
[[no:Revmatologi]]
[[pl:Reumatologia]]
[[pt:Reumatologia]]
[[ru:Ревматология]]
[[sv:Reumatologi]]
[[tr:Romatoloji]]

{{WH}}
{{WS}}

Rheumatoid arthritis

2020-05-09T15:19:02Z

Ayesha A. Khan:

__NOTOC__

'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''

{{Rheumatoid arthritis}}
{{CMG}}; {{AE}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]] , {{MKK}}

{{SK}} [[Rheumatoid disease]]
===Click here for [[The Heart in Rheumatoid Arthritis]]===

==[[Rheumatoid arthritis overview|Overview]]==

==[[Rheumatoid arthritis historical perspective|Historical Perspective]]==

==[[Rheumatoid arthritis classification|Classification]]==

==[[Rheumatoid arthritis pathophysiology|Pathophysiology]]==

==[[Rheumatoid arthritis causes|Causes]]==

==[[Rheumatoid arthritis differential diagnosis|Differentiating Rheumatoid arthritis from Other Diseases]]==

==[[Rheumatoid arthritis epidemiology and demographics|Epidemiology and Demographics]]==

==[[Rheumatoid arthritis risk factors|Risk Factors]]==

==[[Rheumatoid arthritis screening|Screening]]==

==[[Rheumatoid arthritis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==

==Diagnosis==
[[Rheumatoid arthritis diagnostic study of choice |Diagnostic study of choice]] | [[Rheumatoid arthritis history and symptoms|History and Symptoms]] | [[Rheumatoid arthritis physical examination|Physical Examination]] | [[Rheumatoid arthritis laboratory tests|Laboratory Findings]] | [[Rheumatoid arthritis electrocardiogram|Electrocardiogram]] | [[Rheumatoid arthritis x ray|X ray]] | [[Rheumatoid arthritis echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Rheumatoid arthritis CT|CT]] | [[Rheumatoid arthritis MRI|MRI Findings]] | [[Rheumatoid arthritis other imaging findings|Other Imaging Findings]] | [[Rheumatoid arthritis other diagnostic studies|Other Diagnostic Studies]]

==Treatment==
[[Rheumatoid arthritis medical therapy|Medical Therapy]] | [[Rheumatoid arthritis surgical therapy|Surgical Therapy]] | [[Rheumatoid arthritis Primary prevention|Primary prevention]] | [[Rheumatoid arthritis secondary prevention|Secondary prevention]] | [[Rheumatoid arthritis future or investigational therapies|Future or Investigational Therapies]]

==Case Studies==
:[[Rheumatoid arthritis case study 1|Case #1]]

==Related Chapters==
* [[Arthritis]]
* [[Juvenile idiopathic arthritis]]

{{Diseases of the musculoskeletal system and connective tissue}}

[[Category:Aging-associated diseases]]
[[Category:Arthritis]]
[[Category:Autoimmune diseases]]
[[Category:Diseases involving the fasciae]]
[[Category:Rheumatology]]
[[Category:Primary care]]

{{WH}}
{{WS}}

Rheumatoid arthritis

2020-05-09T15:18:09Z

Ayesha A. Khan:

__NOTOC__

'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''

{{Rheumatoid arthritis}}
{{CMG}}; {{AE}} [[User:Ayesha A. Khan|Ayesha A. Khan, MD]] {{MKK}}

{{SK}} [[Rheumatoid disease]]
===Click here for [[The Heart in Rheumatoid Arthritis]]===

==[[Rheumatoid arthritis overview|Overview]]==

==[[Rheumatoid arthritis historical perspective|Historical Perspective]]==

==[[Rheumatoid arthritis classification|Classification]]==

==[[Rheumatoid arthritis pathophysiology|Pathophysiology]]==

==[[Rheumatoid arthritis causes|Causes]]==

==[[Rheumatoid arthritis differential diagnosis|Differentiating Rheumatoid arthritis from Other Diseases]]==

==[[Rheumatoid arthritis epidemiology and demographics|Epidemiology and Demographics]]==

==[[Rheumatoid arthritis risk factors|Risk Factors]]==

==[[Rheumatoid arthritis screening|Screening]]==

==[[Rheumatoid arthritis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==

==Diagnosis==
[[Rheumatoid arthritis diagnostic study of choice |Diagnostic study of choice]] | [[Rheumatoid arthritis history and symptoms|History and Symptoms]] | [[Rheumatoid arthritis physical examination|Physical Examination]] | [[Rheumatoid arthritis laboratory tests|Laboratory Findings]] | [[Rheumatoid arthritis electrocardiogram|Electrocardiogram]] | [[Rheumatoid arthritis x ray|X ray]] | [[Rheumatoid arthritis echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Rheumatoid arthritis CT|CT]] | [[Rheumatoid arthritis MRI|MRI Findings]] | [[Rheumatoid arthritis other imaging findings|Other Imaging Findings]] | [[Rheumatoid arthritis other diagnostic studies|Other Diagnostic Studies]]

==Treatment==
[[Rheumatoid arthritis medical therapy|Medical Therapy]] | [[Rheumatoid arthritis surgical therapy|Surgical Therapy]] | [[Rheumatoid arthritis Primary prevention|Primary prevention]] | [[Rheumatoid arthritis secondary prevention|Secondary prevention]] | [[Rheumatoid arthritis future or investigational therapies|Future or Investigational Therapies]]

==Case Studies==
:[[Rheumatoid arthritis case study 1|Case #1]]

==Related Chapters==
* [[Arthritis]]
* [[Juvenile idiopathic arthritis]]

{{Diseases of the musculoskeletal system and connective tissue}}

[[Category:Aging-associated diseases]]
[[Category:Arthritis]]
[[Category:Autoimmune diseases]]
[[Category:Diseases involving the fasciae]]
[[Category:Rheumatology]]
[[Category:Primary care]]

{{WH}}
{{WS}}

User:Ayesha A. Khan

2020-05-09T15:15:30Z

Ayesha A. Khan: /* Hospital affiliations */

[[User:Ayesha A. Khan|Ayesha A. Khan, MD]]

Dr. Ayesha Khan, MD is an Internist in Cleveland, OH.

==Specialties==
Internist, Rheumatology

==Hospital affiliations==

*Kindred Hospital-Cleveland-Gateway
*Outreach Professional Services Inc
*St. Vincent Charity Medical Center

User:Ayesha A. Khan

2020-05-09T15:13:39Z

Ayesha A. Khan:

[[User:Ayesha A. Khan|Ayesha A. Khan, MD]]

Dr. Ayesha Khan, MD is an Internist in Cleveland, OH.

==Specialties==
Internist, Rheumatology

==Hospital affiliations==
Kindred Hospital-Cleveland-Gateway
Outreach Professional Services Inc
St. Vincent Charity Medical Center