Systemic lupus erythematosus natural history, complications and prognosis: Difference between revisions

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{{CMG}}; {{AE}}{{MIR}}
{{CMG}}; {{AE}}{{MIR}}
==Overview==
==Overview==
Common complications of systemic lupus erythematosus include dermatitis, nephritis and arthiritis. Prognosis is generally poor, and the 10-year mortality rate of patients with systemic lupus erythematosus is approximately 40%. The disease course can be divided into 4 subcategories based on the course of the disease: developmental phase, preclinical phase, clinical phase, and comorbid complication phase.
Common complications of systemic lupus erythematosus include [[dermatitis]], [[nephritis]], and [[arthritis]]. [[Prognosis]] is generally poor, and the 10-year [[mortality rate]] of patients with systemic lupus erythematosus is approximately 40%. The disease's course can be divided into 4 subcategories: developmental phase, preclinical phase, clinical phase, and comorbid complication phase.
==Natural History==
==Natural History==
*Systemic lupus erythematosus (SLE) is an [[autoimmune disease]]. SLE is a disease of waxing and waning, with a lot of flare up episodes. SLE usually develop in the second and third decade of life, although it can develop in any age, and start 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 of the patients.
Systemic lupus erythematosus (SLE) is an [[autoimmune disease]]. Several flare-ups may happen in the course of the disease. SLE usually develops in the second and third decades of life, although it can present at any age. It usually starts 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 course can be divided into 4 subcategories based on the course of the disease:
 
The disease course can be divided into 4 subcategories based on the course of the disease:<ref name="pmid22028590">{{cite journal |vauthors=Iwata Y, Furuichi K, Kaneko S, Wada T |title=The role of cytokine in the lupus nephritis |journal=J. Biomed. Biotechnol. |volume=2011 |issue= |pages=594809 |year=2011 |pmid=22028590 |pmc=3199078 |doi=10.1155/2011/594809 |url=}}</ref><ref name="pmid18305268">{{cite journal |vauthors=Rahman A, Isenberg DA |title=Systemic lupus erythematosus |journal=N. Engl. J. Med. |volume=358 |issue=9 |pages=929–39 |year=2008 |pmid=18305268 |doi=10.1056/NEJMra071297 |url=}}</ref><ref name="pmid1893619">{{cite journal |vauthors=Deguchi Y, Kishimoto S |title=Tumour necrosis factor/cachectin plays a key role in autoimmune pulmonary inflammation in lupus-prone mice |journal=Clin. Exp. Immunol. |volume=85 |issue=3 |pages=392–5 |year=1991 |pmid=1893619 |pmc=1535595 |doi= |url=}}</ref>
 
===== Developmental phase: =====
===== Developmental phase: =====
*[[Genetic mutations]]
*[[Genetic mutations]]
Line 16: Line 17:
*Mostly associated with [[auto-immune]] [[antibody]] production
*Mostly associated with [[auto-immune]] [[antibody]] production
*[[Autoantibodies]] common to other systemic autoimmune diseases
*[[Autoantibodies]] common to other systemic autoimmune diseases
*Proceeds with a more disease-specific clinically overt autoimmune phase
*Proceeds with a more disease-specific clinically overt [[Autoimmune disease|autoimmune phase]]
   
   
===== Clinical phase: =====
===== Clinical phase: =====
*The phase due to damages of the auto-antibodies to the body tissues (mostly related to disease itself)
*The phase due to damages of the [[autoantibodies]] to the body tissues (mostly related to disease itself)
*[[Inflammation]]
*[[Inflammation]]
*Involvement of first organs
*Involvement of first organs
*Flares
*Flares
*Involvement of additional organs
*Involvement of additional organs
*Early damages (e.g. alopecia, fixed erythema, cognitive dysfunction, valvular heart disease, avascular necrosis, tendon rupture, Jaccoud’s arthropathy, and osteoporosis)
*Early damages (e.g. [[alopecia]], [[Erythema|fixed erythema]], [[Cognitive disorder|cognitive dysfunction]], [[valvular heart disease]], [[avascular necrosis]], [[Tendonitis|tendon rupture]], [[Jaccoud's arthropathy|Jaccoud’s arthropathy]], and [[osteoporosis]])
   
   
===== Comorbidity-complication phase =====
===== Comorbidity-complication phase =====
*The phase of damages due to complications of longstanding disease, [[immunosuppressive therapy]], and end organ damages (irreversible damages and complications)
The phase of damages due to complications of longstanding disease, [[immunosuppressive therapy]], and [[end organ damage]] (irreversible damages and complications)
*Infections
*[[Infections]]
*[[Atherosclerosis]]
*[[Atherosclerosis]]
*[[Malignancies]]
*[[Malignancies]]


=== Flare up associated factors: ===
=== Factors associated with flare up: <ref name="pmid26330673">{{cite journal |vauthors=Crow MK, Olferiev M, Kirou KA |title=Identification of Candidate Predictors of Lupus Flare |journal=Trans. Am. Clin. Climatol. Assoc. |volume=126 |issue= |pages=184–96 |year=2015 |pmid=26330673 |pmc=4530671 |doi= |url=}}</ref><ref name="pmid1893619">{{cite journal |vauthors=Deguchi Y, Kishimoto S |title=Tumour necrosis factor/cachectin plays a key role in autoimmune pulmonary inflammation in lupus-prone mice |journal=Clin. Exp. Immunol. |volume=85 |issue=3 |pages=392–5 |year=1991 |pmid=1893619 |pmc=1535595 |doi= |url=}}</ref><ref name="pmid3199078">{{cite journal |vauthors=Josić D, Hofmann W, Habermann R, Schulzke JD, Reutter W |title=Isolation of immunoglobulins and their use in immunoaffinity HPLC |journal=J. Clin. Chem. Clin. Biochem. |volume=26 |issue=9 |pages=559–68 |year=1988 |pmid=3199078 |doi= |url=}}</ref>  ===
* Stress (emotional etc.)
* [[Stress]] (emotional, etc.)
* Sunlight
* [[Sunlight]]
* Ultraviolet light
* [[Ultraviolet]] light
* Infection
* [[Infection]]
* Injuries
* [[Injuries]]
* Surgery
* [[Surgery]]
* Pregnancy
* [[Pregnancy]]
* Abrupt discontinuation of medications
* Abrupt discontinuation of medications
* Treatment noncompliance
* Treatment noncompliance
* Medications
* [[Medications]]
* Immunizations
* [[Immunization|Immunizations]]
* [[Lupus nephritis]]
* Presence of [[Neurological disease|neurologic]] complications
* Presence of [[vasculitis]]
* Elevated [[anti-dsDNA]]
* Low C3 level


==Complications==
==Complications==
Complications that can develop as a result of prolonged activation of systemic lupus erythematosus or the SLE therapy are:
Complications that can develop as a result of prolonged activation of systemic lupus erythematosus or the SLE therapy are:<ref name="pmid23395811">{{cite journal |vauthors=Gurevitz SL, Snyder JA, Wessel EK, Frey J, Williamson BA |title=Systemic lupus erythematosus: a review of the disease and treatment options |journal=Consult Pharm |volume=28 |issue=2 |pages=110–21 |year=2013 |pmid=23395811 |doi=10.4140/TCP.n.2013.110 |url=}}</ref><ref name="pmid24234325">{{cite journal |vauthors=Zubair A, Frieri M |title=Lupus nephritis: review of the literature |journal=Curr Allergy Asthma Rep |volume=13 |issue=6 |pages=580–6 |year=2013 |pmid=24234325 |doi=10.1007/s11882-013-0394-4 |url=}}</ref><ref name="pmid20477476">{{cite journal |vauthors=Torres A, Askari AD, Malemud CJ |title=Cardiovascular disease complications in systemic lupus erythematosus |journal=Biomark Med |volume=3 |issue=3 |pages=239–52 |year=2009 |pmid=20477476 |doi=10.2217/bmm.09.14 |url=}}</ref><ref name="pmid18852220">{{cite journal |vauthors=Cortes S, Chambers S, Jerónimo A, Isenberg D |title=Diabetes mellitus complicating systemic lupus erythematosus - analysis of the UCL lupus cohort and review of the literature |journal=Lupus |volume=17 |issue=11 |pages=977–80 |year=2008 |pmid=18852220 |doi=10.1177/0961203308091539 |url=}}</ref><ref name="pmid18703174">{{cite journal |vauthors=Doria A, Canova M, Tonon M, Zen M, Rampudda E, Bassi N, Atzeni F, Zampieri S, Ghirardello A |title=Infections as triggers and complications of systemic lupus erythematosus |journal=Autoimmun Rev |volume=8 |issue=1 |pages=24–8 |year=2008 |pmid=18703174 |doi=10.1016/j.autrev.2008.07.019 |url=}}</ref><ref name="pmid6127033">{{cite journal |vauthors=Zizic TM, Classen JN, Stevens MB |title=Acute abdominal complications of systemic lupus erythematosus and polyarteritis nodosa |journal=Am. J. Med. |volume=73 |issue=4 |pages=525–31 |year=1982 |pmid=6127033 |doi= |url=}}</ref><ref name="pmid22879850">{{cite journal |vauthors=Cojocaru M, Cojocaru IM, Silosi I, Vrabie CD |title=Manifestations of systemic lupus erythematosus |journal=Maedica (Buchar) |volume=6 |issue=4 |pages=330–6 |year=2011 |pmid=22879850 |pmc=3391953 |doi= |url=}}</ref><ref name="pmid18456233">{{cite journal |vauthors=Clowse ME, Jamison M, Myers E, James AH |title=A national study of the complications of lupus in pregnancy |journal=Am. J. Obstet. Gynecol. |volume=199 |issue=2 |pages=127.e1–6 |year=2008 |pmid=18456233 |pmc=2542836 |doi=10.1016/j.ajog.2008.03.012 |url=}}</ref><ref name="pmid25369438">{{cite journal |vauthors=Bhattacharyya S, Helfgott SM |title=Neurologic complications of systemic lupus erythematosus, sjögren syndrome, and rheumatoid arthritis |journal=Semin Neurol |volume=34 |issue=4 |pages=425–36 |year=2014 |pmid=25369438 |doi=10.1055/s-0034-1390391 |url=}}</ref><ref name="pmid27329649">{{cite journal |vauthors=Alves SC, Fasano S, Isenberg DA |title=Autoimmune gastrointestinal complications in patients with systemic lupus erythematosus: case series and literature review |journal=Lupus |volume=25 |issue=14 |pages=1509–1519 |year=2016 |pmid=27329649 |doi=10.1177/0961203316655210 |url=}}</ref><ref name="pmid12960476">{{cite journal |vauthors=Kang I, Park SH |title=Infectious complications in SLE after immunosuppressive therapies |journal=Curr Opin Rheumatol |volume=15 |issue=5 |pages=528–34 |year=2003 |pmid=12960476 |doi= |url=}}</ref>
 
<small>(Up arrows represent higher frequencies and down arrows represent lower frequencies)</small>
{| class="wikitable"
{| class="wikitable"
!Organ
! style="background: #4479BA; color: #FFFFFF; " |Organ
!Disease
! style="background: #4479BA; color: #FFFFFF; " |Disease
!Description
! style="background: #4479BA; color: #FFFFFF; " |Description
!Frequency
! style="background: #4479BA; color: #FFFFFF; " |Frequency
|-
|-
| rowspan="8" |Gastrointestinal involvement
| rowspan="8" |<small>Gastrointestinal</small>
|[[Dysphagia]]
| style="background: #DCDCDC; " align="center" |[[Dysphagia]]
|
* The most frequent gastrointestinal complaint and is usually due to an underlying esophageal motility disorder, concomitant gastroesophageal reflux disease
|
|
* Usually due to an underlying [[esophageal motility disorder]]
* Concomitant [[gastroesophageal reflux disease]]
|↑↑↑
|-
|-
|[[Peptic ulcer disease]]
| style="background: #DCDCDC; " align="center" |[[Peptic ulcer disease]]
|
* May be due to the disease itself or the effect of SLE treatment
|
|
* Due to:
** The disease itself
** The adverse effect of SLE treatment
|↑
|-
|-
|[[Intestinal pseudo-obstruction|Intestinal pseudo-obstruction]]
| style="background: #DCDCDC; " align="center" |[[Intestinal pseudo-obstruction|Intestinal pseudo-obstruction]]
|
* A rare complication of SLE and lead to mechanical obstruction of the small or large bowel in the absence of an anatomic lesion obstructing the flow of intestinal contents
|
|
* May lead to [[Gastrointestinal obstruction|mechanical obstruction]] of the small or large bowel in the absence of an anatomic lesion
* Obstructing the flow of intestinal contents
|↓↓
|-
|-
|[[Protein-losing enteropathy|Protein-losing enteropathy]]
| style="background: #DCDCDC; " align="center" |[[Protein-losing enteropathy|Protein-losing enteropathy]]
|
* It typically occurs in patients with clinically severe SLE with multi-organ involvement and characterized with the onset of profound edema and hypoalbuminemia in the absence of nephrotic range proteinuria
|
|
* Occurs in patients with clinically severe SLE with multi-organ involvement
* Characterized by:
** [[Edema|Profound edema]]
** [[Hypoalbuminemia]]
* The absence of [[nephrotic]] range [[proteinuria]]
|↓↓
|-
|-
|[[Hepatitis]]
| style="background: #DCDCDC; " align="center" |[[Hepatitis]]
|
* May be due to a wide range of factors including drug-induced damage, steatosis, viral hepatitis, vascular thrombosis, overlap with autoimmune hepatitis, or SLE itself
|
|
* May be due to:
** [[Drug-induced lupus erythematosus|Drug-induced damage]]
** [[Steatosis]]
** [[Viral hepatitis]] (concomitant with SLE)
** [[Thrombosis|Vascular thrombosis]]
** Overlap with [[autoimmune hepatitis]] due to SLE itself
|↑
|-
|-
|[[Acute pancreatitis|Acute pancreatitis]]
| style="background: #DCDCDC; " align="center" |[[Acute pancreatitis|Acute pancreatitis]]
|
* It occurs usually in the setting of active SLE
|
|
* Occurs usually in the setting of active SLE
|↓
|-
|-
|[[Mesenteric vascular occlusion|Mesenteric vasculitis]]
| style="background: #DCDCDC; " align="center" |[[Mesenteric vascular occlusion|Mesenteric vasculitis]]
|
* Most often this involves medium-sized branches of the celiac, superior mesenteric, and inferior mesenteric arteries. Features of mesenteric vasculitis include abdominal pain or gastrointestinal bleeding with intestinal ischemia, infarction, perforation, or pancreatitis
* Primary peritonitis: an infection that develops in the peritoneum mainly due to lupus vasculitis
|
|
* Mostly involve:
** Medium-sized branches of the [[Celiac artery|celiac artery]]
** [[Superior mesenteric artery|Superior mesenteric artery]] 
** [[Inferior mesenteric artery|Inferior mesenteric artery]]
* Features of [[mesenteric]] [[vasculitis]] include:
** [[Abdominal pain]]
** [[Gastrointestinal bleeding]]
** [[Intestinal ischemia]], [[infarction]], [[perforation]]
** [[Pancreatitis]]
** [[SBP|Primary spontaneous peritonitis]]:
*** An [[infection]] that develops in the [[peritoneum]] mainly due to lupus [[vasculitis]]
|↓↓
|-
|-
|[[Acute cholecystitis]]
| style="background: #DCDCDC; " align="center" |[[Acute cholecystitis]]
|
* Due to periarterial fibrosis and acute vasculitis
* It can progress to gangrene, perforation, and sepsis.
|
|
* Due to periarterial [[fibrosis]] and [[Vasculitis|acute vasculitis]]
* May progress to [[gangrene]], [[perforation]], and [[sepsis]]
|↓↓
|-
|-
| rowspan="6" |Pulmonary involvement
| rowspan="6" |<small>Pulmonary</small>
|[[Pleural disease|Pleural disease]]
| style="background: #DCDCDC; " align="center" |[[Pleural disease|Pleural disease]]
|
* [[Pleuritis]] can lead to pleuritic chest pain with or without radiographic evidence of a pleural effusion. Pleuritis is a disease state in which there is inflammation of the [[pleura]], the lining of the [[pleural cavity]], surrounding the [[Lung|lungs]].
* [[Pneumothorax]]: a collection of air within the pleural cavity
|
|
* May lead to:
** [[Pleuritic chest pain]] with or without [[radiographic]] evidence of a [[pleural effusion]]
* [[Inflammation]] of the [[pleura]], the lining of the [[pleural cavity]], surrounding the [[Lung|lungs]]
* [[Pneumothorax]]: a collection of air within the [[pleural cavity]]
|↑
|-
|-
|[[Pneumonitis|Acute pneumonitis]]
| style="background: #DCDCDC; " align="center" |[[Pneumonitis|Acute pneumonitis]]
|
* A rare and fulminant form of diffuse lung injury that generally occurs in previously healthy individuals and has a rapid onset with fever, cough, and shortness of breath
|
|
* Fulminant form of diffuse lung injury
* Characterized by rapid onset with [[fever]], [[cough]], and [[Dyspnea|shortness of breath]]
|↓↓
|-
|-
|[[Pulmonary hemorrhage]]
| style="background: #DCDCDC; " align="center" |[[Pulmonary hemorrhage]]
|
|
* Pulmonary alveolar hemorrhage:
* Pulmonary alveolar hemorrhage:
** A rare complication
** Acutely ill patients with [[hemoptysis]], [[fever]], [[cough]], and [[hypoxemia]]
** Acutely ill patients with hemoptysis, fever, cough, and hypoxemia, and blood loss can be extensive
** Extensive blood loss
** Associated with high mortality rates of 70%–90%
** Associated with high mortality rates of 70%–90%
|
|↓↓
|-
|-
|[[Pulmonary hypertension]]
| style="background: #DCDCDC; " align="center" |[[Pulmonary hypertension]]
|
* An increase in blood pressure in the [[pulmonary artery]] or [[lung]] [[Pulmonary circulation|vasculature]], leading to [[Dypsnea|shortness of breath]], [[dizziness]], [[fainting]], and other symptoms, all of which are exacerbated by exertion
|
|
* Increased pressure in the [[pulmonary artery]] or [[lung]] [[Pulmonary circulation|vasculature]]
* Characterized by [[Dypsnea|shortness of breath]], [[dizziness]], and [[faint]]
|↑
|-
|-
|[[Thromboembolic disease]]
| style="background: #DCDCDC; " align="center" |[[Thromboembolic disease]]
|
* Chronic inflammation during the disease flare ups is an important characteristic in SLE patients that increase the risk of thromboembolic events
|
|
* Chronic [[inflammation]] that increase the risk of [[Thromboembolic disease|thromboembolic events]]
|↑
|-
|-
|Shrinking lung syndrome
| style="background: #DCDCDC; " align="center" |Shrinking lung syndrome
|
* A rare complication of SLE, that can cause dyspnea and shortness of with a reported prevalence of 0.5% of this overall population
|
|
* Characterized by [[dyspnea]] and [[Dyspnea|shortness of breath]] (estimated prevalence approximately 0.5%)
|↓↓
|-
|-
| rowspan="6" |Cardiac involvement
| rowspan="5" |<small>Cardiac</small>
|[[Cardiomegaly]]  
| style="background: #DCDCDC; " align="center" |[[Cardiomegaly]]  
|
|
* Can be a result of:
* Due to:
** [[Myocarditis]]
** [[Myocarditis]]
** [[Libman-Sacks endocarditis]]
** [[Libman-Sacks endocarditis]]
** [[Subacute bacterial endocarditis]]
** [[Subacute bacterial endocarditis]]
** [[Uremia]]
** [[Uremia]]
** [[Pulmonary arterial hypertension]] with right-sided heart failure
** [[Pulmonary arterial hypertension]] with [[right-sided heart failure]]
** Corticosteroid-related [[cardiomyopathy]]
** [[Corticosteroid]]-related [[cardiomyopathy]]
|
|↑↑
|-
|-
|[[Valvular disease]]
| style="background: #DCDCDC; " align="center" |[[Valvular disease]]
|
* Valvular involvement in systemic lupus erythematosus (SLE) is the most prevalent form of heart involvement in SLE
* Immunoglobulin and complement deposition in the valvular structure will lead to different valvular complications include Libman-Sacks vegetations, valve thickening, and valve regurgitation. Involvement of the mitral valve is most frequently encountered
|
|
* Different [[Valvular Diseases|valvular]] complications include:
** [[Libman-Sacks endocarditis|Libman-Sacks vegetations]]
** Valve thickening
** Valve regurgitation
* Mainly due to [[immunoglobulin]] and [[complement]] deposition in valvular structure
* Affect the [[mitral valve]] more frequently
|↑↑
|-
|-
|[[Nonbacterial thrombotic endocarditis]]
| style="background: #DCDCDC; " align="center" |[[Pericardial disease]]
|
* An spectrum of noninfectious lesions of the heart valves that is most commonly seen in advanced malignancy
|
|
* [[Acute pericarditis]]
* [[Pericardial effusion]]
|↓
|-
|-
|[[Pericardial disease]]
| style="background: #DCDCDC; " align="center" |[[Myocarditis]]
|
* The most important way that SLE can affect the heart is through pericardium and by causing an acute percarditis. It usually present as a positional sharp chest pain with radiation to the scapula
|
|
* Characterized by:
** [[Chest pain]]
** [[ST segment elevation]]
** Elevated biomarkers of [[myonecrosis]]
** [[Heart failure]]
** [[Sudden death]]
* [[Myonecrosis]] may happen as a consequence of [[autoimmune]] reaction
|↓
|-
|-
|[[Myocarditis]]
| style="background: #DCDCDC; " align="center" |[[Coronary heart disease|Coronary artery disease]]
|
|
* It may present with [[chest pain]], [[ST segment elevation]], elevated biomarkers of [[myonecrosis]], [[heart failure]], and/ or [[sudden death]]. The inflammatory response especially during disease flare ups cause [[myonecrosis]] either directly or indirectly as part of an [[autoimmune]] reaction
* Mainly as a result of increased risk of [[Atheroma|atheromatous plaques]] due to autoimmune status of SLE
|
|-
|[[Coronary heart disease|Coronary artery disease]]
|
* Myocardial infarction (MI)
* Narrowing of the small blood vessels that supply blood and oxygen to the heart as a result of accumulation of [[Atheroma|atheromatous plaques]] following autoimmune state of SLE
|↑↑
|↑↑
|-
|-
| rowspan="5" |Neurological involvement
| rowspan="5" |<small>Neurological</small>
|[[Cognitive-shifting|Cognitive dysfunction]]
| style="background: #DCDCDC; " align="center" |[[Cognitive-shifting|Cognitive dysfunction]]
|
* The mental status of SLE patients can be temporarily affected by multiple, transient metabolic and systemic processes
|
|
* May be temporarily affected by multiple, transient [[metabolic]] and systemic processes
|↑
|-
|-
|[[Stroke]]
| style="background: #DCDCDC; " align="center" |[[Stroke]]
|
* Small vessel vasculopathy in thebrain during SLE disease is considered as one f the important factors in causing thrombosis and stroke in SLE patients
|
|
* Increase risk of [[Ischemic stroke classification|thrombotic stroke]] due to [[Vasculopathy|small vessel vasculopathy]]
|↓
|-
|-
|[[Seizure|Seizures]]
| style="background: #DCDCDC; " align="center" |[[Seizure|Seizures]]
|
* Increased intracranial pressure can arise from a hypercoagulability state or thrombosis within the cerebral venous and can be the reason of seizures in these patients
|
|
* May happen secondary to [[increased intracranial pressure]]:
** [[Hypercoagulability]] state (due to [[inflammation]])
** [[Thrombosis]] within the [[Cerebral venous sinus thrombosis|cerebral venous]]
|↑
|-
|-
|[[Psychosis]]
| style="background: #DCDCDC; " align="center" |[[Psychosis]]
|
* Psychosis is fairly common in SLE and can indirectly influence cognitive performance as well. The psychosis in SLE patients is mostly accompanied by hallucinations
|
|
* Can indirectly influence [[Cognition|cognitive performance]]
* Mostly accompanied by [[Hallucination|hallucinations]] in SLE
|↑↑
|-
|-
|[[Neuropathies]]
| style="background: #DCDCDC; " align="center" |[[Neuropathies]]
|
* Peripheral neuropathy prevalence is high in SLE and it is mostly related to disease activity
* There is a greater chance for the patients with central nervous system involvement to show manifestation of peripheral neuropathy
* There is a predilection for asymmetric and lower extremities involvement, especially peroneal and sural nerves
|
|
* [[Peripheral neuropathy]]
* Mostly related to disease activity
* [[Central nervous system]] involvement association
* A predilection for asymmetric and [[lower extremities]] involvement, especially [[peroneal]] and [[Sural nerve|sural nerves]]
|↑↑
|-
|-
| rowspan="4" |Musculo-skeletal involvement
| rowspan="4" |<small>Musculoskeletal</small>
|[[Arthritis]]
| style="background: #DCDCDC; " align="center" |[[Arthritis]]
|
|
*Mostly symmetrical and non-erosive
*Mostly symmetrical and non-erosive [[arthritis]]
*Arthralgias
*[[Arthralgias]]
*Effusions
*Effusions
*Decreased range of motion of both small and large joints
*Decreased [[range of motion]] of both small and large joints
*Morning stiffness
*Morning [[stiffness]]
|
|↑↑↑↑
|-
|-
|[[Osteonecrosis]] ([[Avascular necrosis]])
| style="background: #DCDCDC; " align="center" |[[Osteonecrosis]] ([[Avascular necrosis]])
|
* Most common in the femoral head
* Can involve humeral head, tibial plateau, and scaphoid navicular 
* Usually bilateral and is often asymptomatic
* Glucocorticoids treatment is associated with the greatest risk of developing the disease
|
|
* Most common in the [[Femoral|femoral head]]
* Can involve [[Humerus|humeral head]], [[Tibial|tibial plateau]], and scaphoid navicular
* Usually [[bilateral]]
* Often [[asymptomatic]]
* [[Glucocorticoids|Glucocorticoids treatment]] is associated with the greatest risk of developing the disease
|↓
|-
|-
|Subcutaneous nodules
| style="background: #DCDCDC; " align="center" |[[Subcutaneous tissue|Subcutaneous nodules]]
|
* In association with active disease
|
|
* Associated with active disease and flare ups
|↑
|-
|-
|Osteoprosis
| style="background: #DCDCDC; " align="center" |[[Osteoporosis]]
|
|
*Mostly due to [[glucocorticoid]] usage
*Mostly due to [[glucocorticoid]] usage
*Loss of height
*Loss of height
*Sudden back pain
*Sudden [[back pain]]
|
|↑
|-
|-
|Skin disorder
| rowspan="5" |<small>Skin</small>
|[[Cutaneous lupus erythematosus]]
| style="background: #DCDCDC; " align="center" |[[Cutaneous lupus erythematosus]]
|
*Erythema in a malar distribution over the cheeks and nose (but sparing the nasolabial folds), which appears after sun exposure
*
|
|
*[[Erythema]] in a [[malar]] distribution over the cheeks and nose (but sparing the [[nasolabial folds]]), which appears after sun exposure
|↑
|-
|-
| style="background: #DCDCDC; " align="center" |[[Photosensitivity]]
|
|
|Photosensitivity
* Common theme for skin [[lesions]] associated with SLE
|common theme for skin lesions associated with SLE
|↑↑↑
|
|-
|-
| style="background: #DCDCDC; " align="center" |[[Alopecia|Non-scarring alopecia]]
|
|
|Nonscarring alopecia
* May occur at some point during the course of their disease
|may occur at some point during the course of their disease
|↑
|
|-
|-
| style="background: #DCDCDC; " align="center" |Oral and nasal ulcers
|
|
|oral and/or nasal ulcers
* Usually painless
|usually painless
|↑↑
|
|-
|-
| style="background: #DCDCDC; " align="center" |Discoid lesions
|
|
|discoid lesions
* More [[inflammatory]]
|more inflammatory and which have a tendency to scar
* Have a tendency to [[scar]]
|
|↑
|-
|-
|Very rare disorders
| rowspan="2" |<small>Very rare disorders</small>
|Malignancy
| style="background: #DCDCDC; " align="center" |[[Malignancy]]
|
|
|
* [[Non-Hodgkin lymphoma|Non-Hodgkin’s lymphoma]]
* [[Lung cancer]]
* [[Liver mass|Liver cancer]]
* [[Vaginal cancer|Vulvar/vaginal cancer]]
* [[Thyroid cancer]]
|↓↓↓
|-
|-
| style="background: #DCDCDC; " align="center" |[[Diabetes mellitus]]
|
|
|Diabetes mellitus 
* Increase predisposition to:
|
** [[Lupus nephritis]]
|
** [[Peripheral neuropathy]]
|-
** [[Retinal disease]]
|
|↓
|Premature gonadal failure
|
|
|}
|}
==Prognosis==
==Prognosis==
The prognosis of systemic lupus erythematosis is ranging from a benign illness to an extremely rapid progressive disease that can lead to a fulminant organ failure and death. Without treatment, systemic lupus eryhtematosus will result in a very high mortality rate, with a report of more than 60% mortality rate during the mid-20th century. SLE is associated with a 10 year mortality of more than 50% among patient with nephritis. The presence of nephritis is associated with a particularly poor prognosis among patients with SLE. The increase in survival rate of patients and better prognosis 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 improvement in SLE diagnosis have led to better prognosis, the mortality rate among SLE patients is still 5 times more than normal population.
The prognosis of systemic lupus erythematosus ranges from a [[benign]] illness to an extremely rapid progressive disease that can lead to a [[Fulminant|fulminant organ failure]] and death. Without treatment, systemic lupus eryhtematosus 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 increase in [[survival rate]] of patients and better prognosis 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 [[prognosis]], the [[mortality rate]] among SLE patients is still 5 times higher than the normal population.<ref name="pmid24851681">{{cite journal |vauthors=Ren Y, Feng X, Zou Y, Pan W, Wang X, Pan J, Zhang M, Tao J, Zhang Y, Tan K, Li J, Ding X, Qian X, Da Z, Wang M, Chen Z, Sun L |title=[Clinical features and prognosis of patients with lupus nephritis] |language=Chinese |journal=Zhonghua Yi Xue Za Zhi |volume=94 |issue=13 |pages=973–6 |year=2014 |pmid=24851681 |doi= |url=}}</ref><ref name="pmid27307448">{{cite journal |vauthors=Ugarte A, Ruiz-Irastorza G |title=SLE: the changing prognosis |journal=Lupus |volume=25 |issue=12 |pages=1285–7 |year=2016 |pmid=27307448 |doi=10.1177/0961203316652948 |url=}}</ref><ref name="pmid20453401">{{cite journal |vauthors=Matsuyama N, Morimoto S, Tokano Y, Amano H, Nozawa K, Isonuma H, Hashimoto H, Takasaki Y |title=The long-term prognosis of lupus nephritis patients treated with intravenous cyclophosphamide |journal=Intern. Med. |volume=49 |issue=9 |pages=823–8 |year=2010 |pmid=20453401 |doi= |url=}}</ref><ref name="pmid26434992">{{cite journal |vauthors=Sabio JM |title=[Systemic lupus erythematosus today] |language=Spanish; Castilian |journal=Med Clin (Barc) |volume=146 |issue=4 |pages=160–2 |year=2016 |pmid=26434992 |doi=10.1016/j.medcli.2015.08.001 |url=}}</ref>


===== Poor prognostic factors for SLE survival: =====
=== Poor prognostic factors for SLE survival:<ref name="pmid24881804">{{cite journal |vauthors=Lisnevskaia L, Murphy G, Isenberg D |title=Systemic lupus erythematosus |journal=Lancet |volume=384 |issue=9957 |pages=1878–88 |year=2014 |pmid=24881804 |doi=10.1016/S0140-6736(14)60128-8 |url=}}</ref> ===
* Presence of nephritis (especially diffuse proliferative glomerulonephritis)
* Presence of [[nephritis]] (especially diffuse proliferative [[glomerulonephritis]])
* Hypertension
* [[Hypertension]]
* Male sex
* Male sex
* Young age
* Young age
* Older age at presentation
* Older age at presentation
* Low socioeconomic status
* Low socioeconomic status
* Black race: Higher rate of nephritis
* Black race: Higher rate of [[nephritis]]
* Presence of antiphospholipid antibodies
* Presence of [[antiphospholipid antibodies]]
* High overall disease activity
* High overall disease activity


===== Prognosis markers: =====
=== Prognosis markers: <ref name="pmid24881804">{{cite journal |vauthors=Lisnevskaia L, Murphy G, Isenberg D |title=Systemic lupus erythematosus |journal=Lancet |volume=384 |issue=9957 |pages=1878–88 |year=2014 |pmid=24881804 |doi=10.1016/S0140-6736(14)60128-8 |url=}}</ref>===
* Serum anti-dsDNA titres correlated with:
===== Serum anti ds-DNA titres correlated with: =====
** Lupus nephritis
* [[Lupus nephritis]]
** Progression to end-stage renal disease
* Progression to [[end-stage renal disease]]
** Increased disease severity
* Increased disease severity
** Damage or poor survival
* Damage or poor survival
* Antiphospholipid antibodiescorrelated with
 
** Features of the antiphospholipid syndrome (APS) (arterial/ venous thrombosis, fetal loss, thrombocytopenia)
===== Antiphospholipid antibodies correlated with: =====
** CNS involvement (especially cerebrovascular disease)
* Features of the [[Antiphospholipid syndrome|antiphospholipid syndrome (APS)]] 
** Severe lupus nephritis  
* CNS involvement  
** Damage accrual
* Severe [[lupus nephritis]]
** Increase in mortality rate  
* Increase in mortality rate


===== SLE in men compared to women: =====
=== SLE in men compared to women: <ref name="pmid19784840">{{cite journal |vauthors=de Carvalho JF, do Nascimento AP, Testagrossa LA, Barros RT, Bonfá E |title=Male gender results in more severe lupus nephritis |journal=Rheumatol. Int. |volume=30 |issue=10 |pages=1311–5 |year=2010 |pmid=19784840 |doi=10.1007/s00296-009-1151-9 |url=}}</ref>===
* Less photosensitivity
* Less [[photosensitivity]]
* More serositis
* More [[serositis]]
* Older age at diagnosis
* Older age at diagnosis
* Higher 1 year mortality compared to women.
* Higher 1 year mortality compared to women


===== SLE in the elderly (>65) compared to middle age prevalency: =====
=== SLE in the elderly (>65) compared to middle age prevalency: <ref name="pmid24297642">{{cite journal |vauthors=Feng X, Zou Y, Pan W, Wang X, Wu M, Zhang M, Tao J, Zhang Y, Tan K, Li J, Chen Z, Ding X, Qian X, Da Z, Wang M, Sun L |title=Associations of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus |journal=Lupus |volume=23 |issue=3 |pages=327–34 |year=2014 |pmid=24297642 |doi=10.1177/0961203313513508 |url=}}</ref> ===
* Lower incidence of:
* Lower incidence of:
** Malar rash
** [[Malar rash]]
** Photosensitivity
** [[Photosensitivity]]
** Purpura
** [[Purpura]]
** Alopecia
** [[Alopecia]]
** Raynaud’s phenomenon
** [[Raynaud’s phenomenon]]
** Renal system involvement
** [[Nephritis]]
** Central nervous system involvement
** [[Central nervous system]] involvement
* Greater prevalence of:
* Greater prevalence of:
** Serositis
** [[Serositis]]
** Pulmonary involvement
** [[Pulmonary]] involvement
** Sicca symptoms
** [[Sicca syndrome]]
** Musculoskeletal manifestations  
** [[Musculoskeletal]] manifestations
 
==References==
==References==
[[Reflist|2]]
{{Reflist|2}}
<references />

Latest revision as of 11:58, 17 August 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahshid Mir, M.D. [2]

Overview

Common complications of systemic lupus erythematosus include dermatitis, nephritis, and arthritis. Prognosis is generally poor, and the 10-year mortality rate of patients with systemic lupus erythematosus is approximately 40%. The disease's course can be divided into 4 subcategories: developmental phase, preclinical phase, clinical phase, and comorbid complication phase.

Natural History

Systemic lupus erythematosus (SLE) is an autoimmune disease. Several flare-ups may happen in the course of the disease. SLE usually develops in the second and third decades of life, although it can present at any age. It usually starts 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 course can be divided into 4 subcategories based on the course of the disease:[1][2][3]

Developmental phase:
Preclinical phase:
Clinical phase:
Comorbidity-complication phase

The phase of damages due to complications of longstanding disease, immunosuppressive therapy, and end organ damage (irreversible damages and complications)

Factors associated with flare up: [4][3][5]

Complications

Complications that can develop as a result of prolonged activation of systemic lupus erythematosus or the SLE therapy are:[6][7][8][9][10][11][12][13][14][15][16]

(Up arrows represent higher frequencies and down arrows represent lower frequencies)

Organ Disease Description Frequency
Gastrointestinal Dysphagia ↑↑↑
Peptic ulcer disease
  • Due to:
    • The disease itself
    • The adverse effect of SLE treatment
 ↑
Intestinal pseudo-obstruction
  • May lead to mechanical obstruction of the small or large bowel in the absence of an anatomic lesion
  • Obstructing the flow of intestinal contents
 ↓↓
Protein-losing enteropathy ↓↓
Hepatitis ↑
Acute pancreatitis
  • Occurs usually in the setting of active SLE
 ↓
Mesenteric vasculitis ↓↓
Acute cholecystitis ↓↓
Pulmonary Pleural disease ↑
Acute pneumonitis ↓↓
Pulmonary hemorrhage
  • Pulmonary alveolar hemorrhage:
↓↓
Pulmonary hypertension ↑
Thromboembolic disease ↑
Shrinking lung syndrome ↓↓
Cardiac Cardiomegaly ↑↑
Valvular disease ↑↑
Pericardial disease ↓
Myocarditis ↓
Coronary artery disease ↑↑
Neurological Cognitive dysfunction
  • May be temporarily affected by multiple, transient metabolic and systemic processes
 ↑
Stroke ↓
Seizures ↑
Psychosis ↑↑
Neuropathies ↑↑
Musculoskeletal Arthritis ↑↑↑↑
Osteonecrosis (Avascular necrosis) ↓
Subcutaneous nodules
  • Associated with active disease and flare ups
 ↑
Osteoporosis ↑
Skin Cutaneous lupus erythematosus ↑
Photosensitivity
  • Common theme for skin lesions associated with SLE
 ↑↑↑
Non-scarring alopecia
  • May occur at some point during the course of their disease
 ↑
Oral and nasal ulcers
  • Usually painless
 ↑↑
Discoid lesions ↑
Very rare disorders Malignancy ↓↓↓
Diabetes mellitus ↓

Prognosis

The prognosis of systemic lupus erythematosus ranges from a benign illness to an extremely rapid progressive disease that can lead to a fulminant organ failure and death. Without treatment, systemic lupus eryhtematosus 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 increase in survival rate of patients and better prognosis 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 prognosis, the mortality rate among SLE patients is still 5 times higher than the normal population.[17][18][19][20]

Poor prognostic factors for SLE survival:[21]

Prognosis markers: [21]

Serum anti ds-DNA titres correlated with:
Antiphospholipid antibodies correlated with:

SLE in men compared to women: [22]

SLE in the elderly (>65) compared to middle age prevalency: [23]

References

  1. ↑ Iwata Y, Furuichi K, Kaneko S, Wada T (2011). "The role of cytokine in the lupus nephritis". J. Biomed. Biotechnol. 2011: 594809. doi:10.1155/2011/594809. PMC 3199078. PMID 22028590.
  2. ↑ Rahman A, Isenberg DA (2008). "Systemic lupus erythematosus". N. Engl. J. Med. 358 (9): 929–39. doi:10.1056/NEJMra071297. PMID 18305268.
  3. ↑ 3.0 3.1 Deguchi Y, Kishimoto S (1991). "Tumour necrosis factor/cachectin plays a key role in autoimmune pulmonary inflammation in lupus-prone mice". Clin. Exp. Immunol. 85 (3): 392–5. PMC 1535595. PMID 1893619.
  4. ↑ Crow MK, Olferiev M, Kirou KA (2015). "Identification of Candidate Predictors of Lupus Flare". Trans. Am. Clin. Climatol. Assoc. 126: 184–96. PMC 4530671. PMID 26330673.
  5. ↑ Josić D, Hofmann W, Habermann R, Schulzke JD, Reutter W (1988). "Isolation of immunoglobulins and their use in immunoaffinity HPLC". J. Clin. Chem. Clin. Biochem. 26 (9): 559–68. PMID 3199078.
  6. ↑ Gurevitz SL, Snyder JA, Wessel EK, Frey J, Williamson BA (2013). "Systemic lupus erythematosus: a review of the disease and treatment options". Consult Pharm. 28 (2): 110–21. doi:10.4140/TCP.n.2013.110. PMID 23395811.
  7. ↑ Zubair A, Frieri M (2013). "Lupus nephritis: review of the literature". Curr Allergy Asthma Rep. 13 (6): 580–6. doi:10.1007/s11882-013-0394-4. PMID 24234325.
  8. ↑ Torres A, Askari AD, Malemud CJ (2009). "Cardiovascular disease complications in systemic lupus erythematosus". Biomark Med. 3 (3): 239–52. doi:10.2217/bmm.09.14. PMID 20477476.
  9. ↑ Cortes S, Chambers S, Jerónimo A, Isenberg D (2008). "Diabetes mellitus complicating systemic lupus erythematosus - analysis of the UCL lupus cohort and review of the literature". Lupus. 17 (11): 977–80. doi:10.1177/0961203308091539. PMID 18852220.
  10. ↑ Doria A, Canova M, Tonon M, Zen M, Rampudda E, Bassi N, Atzeni F, Zampieri S, Ghirardello A (2008). "Infections as triggers and complications of systemic lupus erythematosus". Autoimmun Rev. 8 (1): 24–8. doi:10.1016/j.autrev.2008.07.019. PMID 18703174.
  11. ↑ Zizic TM, Classen JN, Stevens MB (1982). "Acute abdominal complications of systemic lupus erythematosus and polyarteritis nodosa". Am. J. Med. 73 (4): 525–31. PMID 6127033.
  12. ↑ Cojocaru M, Cojocaru IM, Silosi I, Vrabie CD (2011). "Manifestations of systemic lupus erythematosus". Maedica (Buchar). 6 (4): 330–6. PMC 3391953. PMID 22879850.
  13. ↑ Clowse ME, Jamison M, Myers E, James AH (2008). "A national study of the complications of lupus in pregnancy". Am. J. Obstet. Gynecol. 199 (2): 127.e1–6. doi:10.1016/j.ajog.2008.03.012. PMC 2542836. PMID 18456233.
  14. ↑ Bhattacharyya S, Helfgott SM (2014). "Neurologic complications of systemic lupus erythematosus, sjögren syndrome, and rheumatoid arthritis". Semin Neurol. 34 (4): 425–36. doi:10.1055/s-0034-1390391. PMID 25369438.
  15. ↑ Alves SC, Fasano S, Isenberg DA (2016). "Autoimmune gastrointestinal complications in patients with systemic lupus erythematosus: case series and literature review". Lupus. 25 (14): 1509–1519. doi:10.1177/0961203316655210. PMID 27329649.
  16. ↑ Kang I, Park SH (2003). "Infectious complications in SLE after immunosuppressive therapies". Curr Opin Rheumatol. 15 (5): 528–34. PMID 12960476.
  17. ↑ Ren Y, Feng X, Zou Y, Pan W, Wang X, Pan J, Zhang M, Tao J, Zhang Y, Tan K, Li J, Ding X, Qian X, Da Z, Wang M, Chen Z, Sun L (2014). "[Clinical features and prognosis of patients with lupus nephritis]". Zhonghua Yi Xue Za Zhi (in Chinese). 94 (13): 973–6. PMID 24851681.
  18. ↑ Ugarte A, Ruiz-Irastorza G (2016). "SLE: the changing prognosis". Lupus. 25 (12): 1285–7. doi:10.1177/0961203316652948. PMID 27307448.
  19. ↑ Matsuyama N, Morimoto S, Tokano Y, Amano H, Nozawa K, Isonuma H, Hashimoto H, Takasaki Y (2010). "The long-term prognosis of lupus nephritis patients treated with intravenous cyclophosphamide". Intern. Med. 49 (9): 823–8. PMID 20453401.
  20. ↑ Sabio JM (2016). "[Systemic lupus erythematosus today]". Med Clin (Barc) (in Spanish; Castilian). 146 (4): 160–2. doi:10.1016/j.medcli.2015.08.001. PMID 26434992.
  21. ↑ 21.0 21.1 Lisnevskaia L, Murphy G, Isenberg D (2014). "Systemic lupus erythematosus". Lancet. 384 (9957): 1878–88. doi:10.1016/S0140-6736(14)60128-8. PMID 24881804.
  22. ↑ de Carvalho JF, do Nascimento AP, Testagrossa LA, Barros RT, Bonfá E (2010). "Male gender results in more severe lupus nephritis". Rheumatol. Int. 30 (10): 1311–5. doi:10.1007/s00296-009-1151-9. PMID 19784840.
  23. ↑ Feng X, Zou Y, Pan W, Wang X, Wu M, Zhang M, Tao J, Zhang Y, Tan K, Li J, Chen Z, Ding X, Qian X, Da Z, Wang M, Sun L (2014). "Associations of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus". Lupus. 23 (3): 327–34. doi:10.1177/0961203313513508. PMID 24297642.
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