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| __NOTOC__ | | __NOTOC__ |
| {{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}} Ujjwal Rastogi, MBBS [mailto:urastogi@perfuse.org]
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| {{Polymyalgia rheumatica}} | | {{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}} |
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| ==Overview== | | ==Overview== |
| [[Image:Bodydone.GIF|thumb|200px|In polmyalgia rheumatica (PMR), pain is usually located in the [[shoulders]] and [[hips]].]]
| | 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. |
| '''Polymyalgia rheumatica''' (PMR) - (in Greek meaning “pain in many muscles”) is an inflammatory condition of the [[muscles]], which causes pain or stiffness, usually in the [[neck]], shoulders, and hips. The pain can be very sudden, or can occur gradually over a period of time.
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| Most PMR sufferers wake up in the morning with pain in their [[muscles]]; however, there have been cases in which the patient has developed the pain during the evenings.<ref name="multiple">http://arthritis.webmd.com/polymyalgia-rheumatica-temporal-arteritis Gelfand, Jonathan L MD. "Polymyalgia Rheumatica and Temporal Arteritis." WedMD. 17 Nov. 2007. Cleveland Clinic. 16 Mar. 2008 </ref> Along with this disorder, there have been reports that patients who have polymyalgia rheumatica also have [[temporal arteritis]], which causes inflammation that damages large and medium size [[arteries]]. PMR usually goes away within a year or two after treatment.<ref name="citation2">http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=1 "Polymyalgia Rhuematica." MayoClinic. 17 May 2006. 15 Mar. 2008 </ref>
| | ==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". |
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| ==Causes== | | ==Classification== |
| The cause of this disorder is unknown; however, studies have shown that during this disorder, the [[white blood cells]] in the body attack the lining of muscle joints, causing inflammation.<ref name="citation2">http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=1 "Polymyalgia Rhuematica." MayoClinic. 17 May 2006. 15 Mar. 2008 </ref> Recent studies have found that inherited factors also play a role in the probability that an individual will become sick with polymyalgia rheumatica. Several theories have included viral stimulation of the [[immune system]] in genetically susceptible individuals.<ref name="citation3">http://www.medicinenet.com/polymyalgia_rheumatica/article.htm Shiel, William C. MD, FACP, FACR. "Polymyalgia Rheumatica (PMR) & Giant Cell Arteritis (Temporal Arteritis)." MedicineNet. 3 Mar. 2008. 14 Mar. 2008 </ref>
| | There is no established system for the classification of polymyalgia rheumatica. |
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| Manifestation of [[gaint cell arteritis]] as polymyalgia rheumatica is associated with the increased frequency of [[HLA DR4]].<ref name="pmid3259885">{{cite journal |author=Cid MC, Ercilla G, Vilaseca J, ''et al.'' |title=Polymyalgia rheumatica: a syndrome associated with HLA-DR4 antigen |journal=Arthritis Rheum. |volume=31 |issue=5 |pages=678–82 |year=1988 |month=May |pmid=3259885 |doi= |url=}}</ref>.[[PMR]] and [[GCA]] also share the associated sequence polymorphism encoded by the second hypervariable region(HVR) of the [[HLA DRB1 gene]] unlike [[rheumatoid arthritis]] in which the sequence is encoded by third HVR.<ref name="pmid8147928">{{cite journal |author=Weyand CM, Hunder NN, Hicok KC, Hunder GG, Goronzy JJ |title=HLA-DRB1 alleles in polymyalgia rheumatica, giant cell arteritis, and rheumatoid arthritis |journal=Arthritis Rheum. |volume=37 |issue=4 |pages=514–20 |year=1994 |month=April |pmid=8147928 |doi= |url=}}</ref>. Isolated PMR has positive association with TNFb3, independent of HLA DRB1 association.<ref name="pmid10943865">{{cite journal |author=Mattey DL, Hajeer AH, Dababneh A, ''et al.'' |title=Association of giant cell arteritis and polymyalgia rheumatica with different tumor necrosis factor microsatellite polymorphisms |journal=Arthritis Rheum. |volume=43 |issue=8 |pages=1749–55 |year=2000 |month=August |pmid=10943865 |doi=10.1002/1529-0131(200008)43:8<1749::AID-ANR11>3.0.CO;2-K |url=}}</ref>
| | ==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. |
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| Several [[viruses]] are thought to be linked to polymyalgia rheumatica,including the [[adenovirus]], which causes respiratory infections; the human parvovirus B19, an infection that affects children; and the human parainfluenza virus..<ref name="citation2">http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=1 "Polymyalgia Rhuematica." MayoClinic. 17 May 2006. 15 Mar. 2008 </ref>
| | ==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]]. |
| == Differential Diagnosis ==
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| * [[Rheumatoid arthritis]] - Polymyalgia rheumatica and late onset rheumatoid arthritis can initially present with similar clincal features like synovitis. These patients are treated initially as PMR with gluococorticoids. RA treatment is started when there is no improvement or when it evolves into charecteristic RA or when there is a persistently raised plasma viscosity.<ref name="pmid18980958">{{cite journal |author=Pease CT, Haugeberg G, Montague B, ''et al.'' |title=Polymyalgia rheumatica can be distinguished from late onset rheumatoid arthritis at baseline: results of a 5-yr prospective study |journal=Rheumatology (Oxford) |volume=48 |issue=2 |pages=123–7 |year=2009 |month=February |pmid=18980958 |doi=10.1093/rheumatology/ken343 |url=}}</ref> <ref name="pmid15940765">{{cite journal |author=Pease CT, Haugeberg G, Morgan AW, Montague B, Hensor EM, Bhakta BB |title=Diagnosing late onset rheumatoid arthritis, polymyalgia rheumatica, and temporal arteritis in patients presenting with polymyalgic symptoms. A prospective longterm evaluation |journal=J. Rheumatol. |volume=32 |issue=6 |pages=1043–6 |year=2005 |month=June |pmid=15940765 |doi= |url=}}</ref>
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| * [[Polymyositis]] and Dermatomyositis - Patients with dermatomyositis or polymyositis present with tenderness and weakness of proximal muscles, while PMR patients present with pain and stiffness prominently. This differentiation may be difficult in elderly patients. Proper history,complete physical examination, ESR,creatine kinase levels and muscle biopsy help in establishing proper diagnosis.<ref name="pmid21040663">{{cite journal |author=Sørensen CD, Hansen LH, Hørslev-Petersen K |title=[Myositis as differential diagnosis in polymyalgia rheumatica] |language=Danish |journal=Ugeskr. Laeg. |volume=172 |issue=42 |pages=2899–900 |year=2010 |month=October |pmid=21040663 |doi= |url=}}</ref> <ref name="pmid2042988">{{cite journal |author=Hopkinson ND, Shawe DJ, Gumpel JM |title=Polymyositis, not polymyalgia rheumatica |journal=Ann. Rheum. Dis. |volume=50 |issue=5 |pages=321–2 |year=1991 |month=May |pmid=2042988 |pmc=1004419 |doi= |url=}}</ref>
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| * Malignancy ([[myeloma]], others) - Patients with malignancy sometimes present with PMR like symptoms and have poor response to steroid therapy<ref name="pmid3774196">{{cite journal |author=Manganelli P, Borghi L, Coruzzi P, Novarini A, Ambanelli U |title=[Paraneoplastic polymyalgia rheumatica. Case contribution] |language=Italian |journal=Minerva Med. |volume=77 |issue=38 |pages=1739–41 |year=1986 |month=October |pmid=3774196 |doi= |url=}}</ref>. This is in fact paraneoplastic syndrome presenting as PMR.<ref name="pmid19562970">{{cite journal |author=Kwiatkowska B, Filipowicz-Sosnowska A |title=[Polymyalgia rheumatica mimicking neoplastic disease--significant problem in elderly patients] |language=Polish |journal=Pol. Arch. Med. Wewn. |volume=118 Suppl |issue= |pages=47–9 |year=2008 |pmid=19562970 |doi= |url=}}</ref>
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| *[[Fibromyalgia]] - [[Fibromyalgia]] is commonly presented in age groups 20-50 years and patients have characteristic tender points. The active phase protiens and [[ESR]] are normal unlike [[PMR]].
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| *[[Hyperparathyroidism]] - [[Hyperparathyroidism]] presents with proximal stiffness and bone pain with elevated [[parathyroid hormone]] levels and often [[calcium]] levels without elevation of [[ESR]] levels.
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| * Chronic infection ([[subacute bacterial endocarditis]] ([[SBE]])) - Rheumatologic symptoms seen in [[infective endocarditis]] can present a clinical picture suggesting [[polymyalgia rheumatica]] hindering the correct diagnosis.<ref name="pmid16859595">{{cite journal |author=Auzary C, Le Thi Huong D, Delarbre X, ''et al.'' |title=Subacute bacterial endocarditis presenting as polymyalgia rheumatica or giant cell arteritis |journal=Clin. Exp. Rheumatol. |volume=24 |issue=2 Suppl 41 |pages=S38–40 |year=2006 |pmid=16859595 |doi= |url=}}</ref>
| | ==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]]. |
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| * [[Hypothyroidism]] - These patients have signs like muscle and joint pain and weakness similar to [[PMR]]. Delayed relaxation of deep tendon reflexes is seen in [[hypothyroidism]] with elevated [[TSH]] levels and low [[T4]] levels.
| | ==Epidemiology and Demographics== |
| *[[Remitting seronegative symmetrical synovitis with pitting edema]] - [[RS3PE]] presents with symmetrical [[synovitis]] and [[pitting edema]],usually in patients over 50 years of age and lack [[rheumatoid factor]]. The symptoms are commonly manifested distally unlike [[PMR]].
| | 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. |
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| == Epidemiology and Demographics ==
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| * Mean age at onset ~ 70 (range 50-90)
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| * F:M ratio = 2:1
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| ==Risk Factors== | | ==Risk Factors== |
| There are no certain circumstances for which an individual will get [[polymyalgia rheumatica]], but there are a few factors that show a relationship with the disorder.
| | 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. |
| *Usually affects adults over the age of 50<ref name="multiple">http://arthritis.webmd.com/polymyalgia-rheumatica-temporal-arteritis Gelfand, Jonathan L MD. "Polymyalgia Rheumatica and Temporal Arteritis." WedMD. 17 Nov. 2007. Cleveland Clinic. 16 Mar. 2008 </ref>
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| *The average age of a person who has PMR is about 70 years old<ref name="multiple">http://arthritis.webmd.com/polymyalgia-rheumatica-temporal-arteritis Gelfand, Jonathan L MD. "Polymyalgia Rheumatica and Temporal Arteritis." WedMD. 17 Nov. 2007. Cleveland Clinic. 16 Mar. 2008 </ref>
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| *Women are twice as likely to get[[PMR]] as men<ref name="multiple">http://arthritis.webmd.com/polymyalgia-rheumatica-temporal-arteritis Gelfand, Jonathan L MD. "Polymyalgia Rheumatica and Temporal Arteritis." WedMD. 17 Nov. 2007. Cleveland Clinic. 16 Mar. 2008 </ref>
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| *The vast majority of people affected are white<ref name="multiple">http://arthritis.webmd.com/polymyalgia-rheumatica-temporal-arteritis Gelfand, Jonathan L MD. "Polymyalgia Rheumatica and Temporal Arteritis." WedMD. 17 Nov. 2007. Cleveland Clinic. 16 Mar. 2008 </ref>
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| *50% of people with [[temporal arteritis]] also have [[polymyalgia rheumatica]]<ref name="multiple">http://arthritis.webmd.com/polymyalgia-rheumatica-temporal-arteritis Gelfand, Jonathan L MD. "Polymyalgia Rheumatica and Temporal Arteritis." WedMD. 17 Nov. 2007. Cleveland Clinic. 16 Mar. 2008 </ref>
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| *Smoking, sun exposure, infections, [[nulliparity]] are also considered risk factors.<ref name="pmid10948749">{{cite journal |author=Cimmino MA, Zaccaria A |title=Epidemiology of polymyalgia rheumatica |journal=Clin. Exp. Rheumatol. |volume=18 |issue=4 Suppl 20 |pages=S9–11 |year=2000 |pmid=10948749 |doi= |url=}}</ref>
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| == History and Symptoms == | | == Screening == |
| There are a wide range of symptoms that indicate if a person has polymyalgia rheumatica. The symptoms include pain and stiffness in the [[muscles]], usually in the [[neck]], [[shoulders]], and [[hips]]. The pain is moderate to severe, and may inhibit the activity of the person. These symptoms usually occur in the morning, or after sleeping. [[Fatigue]] and [[lack of appetite]] are also signs of polymyalgia rheumatica. [[Lack of appetite]] could lead to unintentional [[weight loss]]. [[Anemia]] is another sign of polymyalgia rheumatica. An overall [[feeling of illness]] and a slight [[fever]] are also signs of this disorder.<ref name="citation2">http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=1 "Polymyalgia Rhuematica." MayoClinic. 17 May 2006. 15 Mar. 2008 </ref> | | There is insufficient evidence to recommend routine screening for polymyalgia rheumatica. |
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| Studies have shown that about 15% of people who are diagnosed with polymyalgia rheumatica also have [[temporal arteritis]], and about 50% of people with [[temporal arteritis]] have polymyalgia rheumatica. Some symptoms of [[temporal arteritis]] include severe[[headaches]], scalp tenderness, [[jaw]] or facial soreness, distorted vision or aching in the limbs caused by decreased blood flow, and [[fatigue]].<ref name="multiple">http://arthritis.webmd.com/polymyalgia-rheumatica-temporal-arteritis Gelfand, Jonathan L MD. "Polymyalgia Rheumatica and Temporal Arteritis." WedMD. 17 Nov. 2007. Cleveland Clinic. 16 Mar. 2008 </ref>
| | ==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]]. |
| * Symmetric aching/stiffness of axial & proximal limb musculature
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| *:* Shoulder girdle, neck, hip girdle
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| *:* Morning stiffness, gelling
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| * Muscle strength intact, though may be limited by pain
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| * [[Synovitis]] in knees, wrists, sterno-clavicular (SC) joints, hips/shoulders
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| * Distal extremity swelling ([[tenosynovitis]])
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| * [[Malaise]], [[fatigue]], [[weight loss]] in > 50%
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| ==== Relation to Giant Cell Arteritis (GCA) ====
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| * 25% of patients with GCA have PMR as presenting symptom
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| * 40-60% of patients with GCA have PMR during disease course
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| * 10-15% of patients with PMR have GCA by temporal artery (TA) biopsy
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| <br>
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| {|
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| |-style="background:silver; color:black"
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| | '''''Symptoms GCA''''' || || '''''Signs GCA''''' ||
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| |-style="background:silver; color:black"
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| | '''Headache''' || '''68%''' || '''Decreased TA pulsations''' || '''46%'''
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| |- style="background:silver; color:black"
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| | '''Jaw claudication''' || '''45%''' || '''Fever''' || '''42%'''
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| |-style="background:silver; color:black"
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| | '''Transient visual symptoms''' || '''16%''' || '''Tenderness over temporal artery''' || '''27%'''
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| |-style="background:silver; color:black"
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| | '''Fixed visual loss''' || '''14%''' || '''Nodular or swollen scalp arteries''' || '''23%'''
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| |-style="background:silver; color:black"
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| | '''Dysphagia ''' || '''8%''' || '''Large artery bruits ''' || '''21%'''
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| |-style="background:silver; color:black"
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| | '''Tongue claudication''' || '''6%''' || '''Ophthalmoscopic abnormalities''' || '''18%'''
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| |-style="background:silver; color:black"
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| | || || '''Visual loss''' || '''14%'''
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| |}
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| ==Diagnosis== | | ==Diagnosis== |
| There is no specific test to diagnose [[polymyalgia rheumatica]]. There are many other diseases which cause [[inflammation]] and pain in [[muscles]], but there are a few tests that can help narrow down the cause of the pain. Limitation in shoulder motion, or swelling of the joints in the wrists or hands are noted by the doctor.<ref name="citation3">http://www.medicinenet.com/polymyalgia_rheumatica/article.htm Shiel, William C. MD, FACP, FACR. "Polymyalgia Rheumatica (PMR) & Giant Cell Arteritis (Temporal Arteritis)." MedicineNet. 3 Mar. 2008. 14 Mar. 2008 </ref> The doctor will assess the patient’s pain, and may perform one of the following tests to determine if [[polymyalgia rheumatica]] is indeed the cause of the pain.<ref name="citation2">http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=1 "Polymyalgia Rhuematica." MayoClinic. 17 May 2006. 15 Mar. 2008 </ref>
| | ===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). |
| One test that is usually performed is the [[erythrocyte sedimentation rate]] test (also known as the [[ESR]] or [[SED rate]]), which is a [[blood test]] that checks the [[erythrocyte sedimentation rate]]. This test measures how fast the patient’s red blood cells settle in a test tube. The faster the blood cells settle, the higher the [[SED rate]], which means that there is [[inflammation]]. Because many conditions can cause an elevated [[SED rate]], this test alone is not a good determinant if a person has with [[polymyalgia rheumatica]].<ref name="citation2">http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=1 "Polymyalgia Rhuematica." MayoClinic. 17 May 2006. 15 Mar. 2008 </ref>
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| Another test that checks the level of [[C-reactive protein]] in the blood may also be conducted. [[C-reactive protein]] is produced by the liver in response to an injury or infection. People with [[polymyalgia rheumatica]] usually have high levels of [[C-reactive protein]], which is a response due to inflammation.<ref name="citation2">http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=1 "Polymyalgia Rhuematica." MayoClinic. 17 May 2006. 15 Mar. 2008 </ref>
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| Because people with polymyalgia rheumatica are sometimes associated with [[temporal arteritis]], doctors may perform a test to determine if a person has [[temporal arteritis]]. A sample from the scalp artery in the temple is taken and examined under a microscope. If a person is positive for [[temporal arteritis]], the doctor may prescribe a medicine that treats both polymyalgia rheumatica and [[temporal arteritis]].<ref name="citation2">http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=1 "Polymyalgia Rhuematica." MayoClinic. 17 May 2006. 15 Mar. 2008 </ref>
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| As a summary;
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| * Age ≥ 50 at onset
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| * Symtoms in ≥ 2/3 areas (shoulder girdle, hip girdle, neck) x ≥ 1 month
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| * [[Erythrocyte sedimentation rate (ESR)]] ≥ 40 mm/hr
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| * Exclusion of alternative diagnosis
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| 2012 provisional criteria for [[Polymyalgia rheumatica]] by [[EULAR/ACR]] : patient ><u></u>50 years old presenting with bilateral shoulder pain, not explained by an alternative pathology can be classified a [[PMR]] in the presence of morning stiffness >45 minutes, elevated [[CRP]] and/or [[ESR]] and new hip pain.<ref name="pmid22388996">{{cite journal |author=Dasgupta B, Cimmino MA, Maradit-Kremers H, ''et al.'' |title=2012 provisional classification criteria for polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative |journal=Ann. Rheum. Dis. |volume=71 |issue=4 |pages=484–92 |year=2012 |month=April |pmid=22388996 |pmc=3298664 |doi=10.1136/annrheumdis-2011-200329 |url=}}</ref>
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| == Laboratory Findings ==
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| * Mild normochromic, normocytic anemia (during active phase)
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| * [[White blood cell]] ([[WBC]]) normal, [[platelets]] often increased
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| * Markedly elevated [[ESR]]
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| * [[Antinuclear antibody]] (ANA), [[rheumatoid factor]] (RF) usually negative
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| *Elevation of [[alkaline phosphatase]] of [[liver]] origin is seen in one third to half of patients with [[PMR]] associated with [[GCA]].<ref name="pmid1807822">{{cite journal |author=Kyle V |title=Laboratory investigations including liver in polymyalgia rheumatica/giant cell arteritis |journal=Baillieres Clin Rheumatol |volume=5 |issue=3 |pages=475–84 |year=1991 |month=December |pmid=1807822 |doi= |url=}}</ref>
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| * [[Creatine kinase]] (CK), aldolase, [[electromyogram]] (EMG), [[muscle biopsy]] all normal
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| === Other Diagnostic Studies ===
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| ==== Temporal Artery Biopsy ====
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| * Not indicated if no symptoms suggestive of [[GCA]]
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| * Negative predictors of [[GCA]]
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| *:* Age < 70
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| *:* Absence of headache or jaw [[claudication]]
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| *:* Clinically normal temporal arteries
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| *:* 3 negative predictors--1.7% risk [[GCA]]
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| *:* If ≤ 2 negative predictors--55% risk [[GCA]]
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| * Can still demonstrates [[arteritis]] after 2-4 weeks of [[steroid]] treatment
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| ==== Ultrasound and MRI ====
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| *[[Ultrasonography]] is effective in confirming bilateral [[subacromial]] and [[subdeltoid bursitis]] in [[PMR]] patients.<ref name="pmid11361188">{{cite journal |author=Cantini F, Salvarani C, Olivieri I, ''et al.'' |title=Shoulder ultrasonography in the diagnosis of polymyalgia rheumatica: a case-control study |journal=J. Rheumatol. |volume=28 |issue=5 |pages=1049–55 |year=2001 |month=May |pmid=11361188 |doi= |url=}}</ref>
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| *[[MRI]] is used in the assessment of [[hip synovitis]] and [[iliopsoas bursitis]]. It is sensitive than [[ultrasonography].<ref name="pmid16095113">{{cite journal |author=Cantini F, Niccoli L, Nannini C, ''et al.'' |title=Inflammatory changes of hip synovial structures in polymyalgia rheumatica |journal=Clin. Exp. Rheumatol. |volume=23 |issue=4 |pages=462–8 |year=2005 |pmid=16095113 |doi= |url=}}</ref>
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| *[[MRI]] and [[Ultrasound]] of the shoulder facilitate the proper diagnosis in patients with the typical proximal symptoms of [[PMR]] with normal [[ESR]] values.<ref name="pmid11352249">{{cite journal |author=Cantini F, Salvarani C, Olivieri I, ''et al.'' |title=Inflamed shoulder structures in polymyalgia rheumatica with normal erythrocyte sedimentation rate |journal=Arthritis Rheum. |volume=44 |issue=5 |pages=1155–9 |year=2001 |month=May |pmid=11352249 |doi=10.1002/1529-0131(200105)44:5<1155::AID-ANR198>3.0.CO;2-N |url=}}</ref>
| | ===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. |
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| *[[Ultrasound]] is useful in monitoring the patients' response to the [[corticosteroid]] treatment and detects any subclinical [[inflammation]].<ref name="pmid19773289">{{cite journal |author=Jiménez-Palop M, Naredo E, Humbrado L, ''et al.'' |title=Ultrasonographic monitoring of response to therapy in polymyalgia rheumatica |journal=Ann. Rheum. Dis. |volume=69 |issue=5 |pages=879–82 |year=2010 |month=May |pmid=19773289 |doi=10.1136/ard.2009.113555 |url=}}</ref>.[[Power Doppler ultrasound]] is useful in detecting patients with high [[inflammation]] who have a high risk of relapses or recurrences.<ref name="pmid19808693">{{cite journal |author=Macchioni P, Catanoso MG, Pipitone N, Boiardi L, Salvarani C |title=Longitudinal examination with shoulder ultrasound of patients with polymyalgia rheumatica |journal=Rheumatology (Oxford) |volume=48 |issue=12 |pages=1566–9 |year=2009 |month=December |pmid=19808693 |doi=10.1093/rheumatology/kep286 |url=}}</ref>
| | ===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. |
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| ==== PET Scan ==== | | ===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. |
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| [[PET scan]] has no proven clinical value in patient care. [[18-fluorodeoxyglucose]] accumulation in the [[blood vessels]] suggests [[arteritis]],mostly in the large vessels. [[Region of index]] analysis is sensitive tool in detecting such [[inflammation]].<ref name="pmid15194587">{{cite journal |author=Moosig F, Czech N, Mehl C, ''et al.'' |title=Correlation between 18-fluorodeoxyglucose accumulation in large vessels and serological markers of inflammation in polymyalgia rheumatica: a quantitative PET study |journal=Ann. Rheum. Dis. |volume=63 |issue=7 |pages=870–3 |year=2004 |month=July |pmid=15194587 |pmc=1755055 |doi=10.1136/ard.2003.011692 |url=}}</ref>[[FDG]] focal uptake is seen in [[ligament]] [[inflammation]].<ref name="pmid22475905">{{cite journal |author=Adams H, Raijmakers P, Smulders Y |title=Polymyalgia rheumatica and interspinous FDG uptake on PET/CT |journal=Clin Nucl Med |volume=37 |issue=5 |pages=502–5 |year=2012 |month=May |pmid=22475905 |doi=10.1097/RLU.0b013e3182485098 |url=}}</ref>
| | === Electrocardiogram === |
| | There are no EKG findings associated with polymyalgia rheumatica. |
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| ==Treatment and Medication== | | ===X Ray=== |
| Anti-inflammatory medicine such as [[aspirin]] or [[ibuprofen]] is typically prescribed to treat mild cases of [[polymyalgia rheumatica]]. For more serious cases, with extreme pain and discomfort, [[steroids]] are prescribed to patients. [[Prednisone]] is the typical [[steroid]] used to treat [[polymyalgia rheumatica]]. The [[steroids]] are normally distributed in low doses (10-15 mg per day), and results are usually seen within the first few days of taking the medication.<ref name="citation4">http://www.rheumatology.org/public/factsheets/pmr_new2.asp "POLYMYALGIA RHEUMATICA." American College of Rheumatology. June 2006. American College of Rheumatology. 11 Mar. 2008 </ref> The patient's SED rate is monitored throughout the medication process, and other [[blood tests]] are conducted to make sure the patient does not experience any side effects from the treatment. Once the SED rate is back to normal, the patient will receive lower doses of the [[steroids]] in order to avoid any long term health effects from the [[steroids]].<ref name="citation2">http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=1 "Polymyalgia Rhuematica." MayoClinic. 17 May 2006. 15 Mar. 2008 </ref> [[Intramuscular]] [[methylprednisolone]](40-120 mg)has similar effects like oral [[corticosteroids]] and the cumulative steroid dose is also less.<ref name="pmid1768166">{{cite journal |author=Dasgupta B, Gray J, Fernandes L, Olliff C |title=Treatment of polymyalgia rheumatica with intramuscular injections of depot methylprednisolone |journal=Ann. Rheum. Dis. |volume=50 |issue=12 |pages=942–5 |year=1991 |month=December |pmid=1768166 |pmc=1004588 |doi= |url=}}</ref>
| | There are no x ray findings associated with polymyalgia rheumatica. |
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| Some side effects from the [[steroids]] may occur. Studies have shown that [[steroids]] increase the patient’s [[blood pressure]]. For this reason, the patient’s [[blood pressure]] is monitored throughout the treatment process. Also, the [[steroids]] lower the patient’s [[immune system]], making them more susceptible to [[infection]]. The doctor should be notified of any signs of sickness.<ref name="citation2">http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=1 "Polymyalgia Rhuematica." MayoClinic. 17 May 2006. 15 Mar. 2008 </ref> [[Methylprednisolone]] has better side effect profile with respect to fracture rate and weight gain.<ref name="pmid10948765">{{cite journal |author=Li C, Dasgupta B |title=Corticosteroids in polymyalgia rheumatica--a review of different treatment schedules |journal=Clin. Exp. Rheumatol. |volume=18 |issue=4 Suppl 20 |pages=S56–7 |year=2000 |pmid=10948765 |doi= |url=}}</ref> Prophylaxis for [[osteoporosis]] with [[calcium]] and [[vitamin D]] should be started along with [[steroid]] therapy.
| | === 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. |
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| Along with medical treatment, patients can increase their chances of recovery by exercising and eating healthy foods. [[Exercise]] will help strengthen the weak [[muscles]], and help to prevent weight gain. A healthy diet will help to keep a strong [[immune system]], and also help build strong muscles and bones.<ref name="citation2">http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=1 "Polymyalgia Rhuematica." MayoClinic. 17 May 2006. 15 Mar. 2008 </ref>
| | ===CT Scan=== |
| | There are no CT findings associated with polymyalgia rheumatica. |
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| Treatment lasts as long as needed; however, it normally takes patients several years to get off of the [[steroids]]. The symptoms may come back when the dosage is lowered.<ref name="citation3">http://www.medicinenet.com/polymyalgia_rheumatica/page2.htm#6whatis Shiel, William C. MD, FACP, FACR. "Polymyalgia Rheumatica (PMR) & Giant Cell Arteritis (Temporal Arteritis)." MedicineNet. 3 Mar. 2008. 14 Mar. 2008 </ref>
| | ===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. |
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| === Acute Pharmacotherapies === | | ===Other Imaging Findings=== |
| * [[NSAID]]s helpful in mild disease
| | There are no other imaging findings associated with polymyalgia rheumatica. |
| * [[Glucocorticoids]] if no response to [[non-steriod anti inflammatory drug]]s ([[NSAID]]s)
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| *:* [[Prednisone]] 5-20 mg every day--rapid improvement in symptoms
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| *:* Start taper after symptoms remit and ESR returns to normal (2-4 weeks)
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| *:* Gradual dose reduction (eg, by 2.5 mg) q 1-4 weeks until dose = 5-10 mg every day
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| *:* Even ''slower'' taper once dose < 5-10 mg (reduce dose by 1 mg q month)
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| * Monitor for relapse--occurs in 25-50%, usually because taper too rapid
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| * Careful observation for signs of [[arteritis]]
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| === Other therapies === | | ===Other Diagnostic Studies=== |
| [[Glucocorticoids]] are the most effective medication for [[polymyalgia rheumatica]]. Other medications are considered in [[steroid]] resistant cases i.e no good response with 20mg/day [[prednisone]], atypical cases and in patients at high risk for [[steroid]] related toxicity. | | 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]]. |
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| *[[Methotrexate]] is the commonly used steroid sparing agent.[[Prednisone]] plus [[methotrexate]] treatment is associated with shorter [[prednisone]] treatment.<ref name="pmid15466766">{{cite journal |author=Caporali R, Cimmino MA, Ferraccioli G, ''et al.'' |title=Prednisone plus methotrexate for polymyalgia rheumatica: a randomized, double-blind, placebo-controlled trial |journal=Ann. Intern. Med. |volume=141 |issue=7 |pages=493–500 |year=2004 |month=October |pmid=15466766 |doi= |url=}}</ref>
| | ==Treatment== |
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| *[[Infliximab]] use in [[[PMR]] has not been proved beneficial and it may be harmful.<ref name="pmid17470831">{{cite journal |author=Salvarani C, Macchioni P, Manzini C, ''et al.'' |title=Infliximab plus prednisone or placebo plus prednisone for the initial treatment of polymyalgia rheumatica: a randomized trial |journal=Ann. Intern. Med. |volume=146 |issue=9 |pages=631–9 |year=2007 |month=May |pmid=17470831 |doi= |url=}}</ref>
| | ===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. |
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| *[[Etanercept]] may be safe and useful in relapsing [[PMR]]. It is modestly effective in [[PMR]] associated with [[giantcell arteritis]] than in isolated [[PMR]].Trials are still in progress to determine the benefit and the differences in response.
| | ===Surgery=== |
| | Surgical intervention is not recommended for the management of polymyalgia rheumatica. |
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| [[EULAR]] response criteria for [[PMR]] comprise a set of core markers for monitoring therapeutic response which include :
| | ===Primary Prevention=== |
| | There are no established measures for the primary prevention of polymyalgia rheumatica. |
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| *[[ESR]]
| | ===Secondary Prevention=== |
| | | There are no established measures for the secondary prevention of polymyalgia rheumatica. |
| *[[CRP]]
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| *Visual analogue scale of patient's pain
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| *[[Physician's global assessment]]
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| *[[Morning stiffness]]
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| *Ability to elevate the [[upper limbs]].
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| A disease activity score <7 indicates low activity, 7-17 suggest medium disease activity, >17 indicates high disease activity.<ref name="pmid16823992">{{cite journal |author=Nothnagl T, Leeb BF |title=Diagnosis, differential diagnosis and treatment of polymyalgia rheumatica |journal=Drugs Aging |volume=23 |issue=5 |pages=391–402 |year=2006 |pmid=16823992 |doi= |url=}}</ref>
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| ==References==
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| {{reflist|2}}
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| ==External links== | | ==External links== |
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| * [http://www.rheumatology.org/public/factsheets/pmr_new2.asp Patient Education - Polymyalgia Rheumatica] - American College of Rheumatology | | * [http://www.rheumatology.org/public/factsheets/pmr_new2.asp Patient Education - Polymyalgia Rheumatica] - American College of Rheumatology |
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| {{Diseases of the musculoskeletal system and connective tissue}}
| | [[Category:Medicine]] |
| {{WikiDoc Help Menu}}
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| {{WikiDoc Sources}}
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| [[Category:Disease]] | |
| [[Category:Rheumatology]] | | [[Category:Rheumatology]] |
| | [[Category:Up-To-Date]] |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Ayesha A. Khan, MD[3] Ujjwal Rastogi, MBBS [4]; Rim Halaby, M.D. [5]; Ahmed Elsaiey, MBBCH [6]
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, shoulders, upper arms, hip, and thighs. Although myalgia is one of the symptoms of PMR, there is no inflammation of the muscles; 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 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 inflammatory disease of the articular and periarticular structures of the cervical region, shoulder girdle and 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 infections such as parainfluenza virus type 1, mycoplasma pneumoniae, chlamydia pneumoniae, and parvovirus B19. In addition, histological examinations of synovial 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 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 inflammatory joint lining attack, viral infections, and 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, 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 joints and limbs, including the neck, the shoulder girdle, the pelvic girdle, the lower back, and the 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 joints. Ophthalmoscopic exams in patients with polymyalgia rheumatica associated with 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