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Polymyalgia rheumatica - Skin
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Polymyalgia rheumatica - Skin

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Contributors: Annie Yang MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Polymyalgia rheumatica (PMR) is a relatively common rheumatological condition that primarily affects individuals over the age of 50. PMR is characterized by neck and bilateral shoulder stiffness and pain with involvement of the upper arms, thighs, and hips. Women are twice as likely as men to be affected.

The exact etiology of PMR is undetermined, but it is believed to be in the same spectrum of disease as giant cell arteritis (GCA) because the same family of HLA serotypes, HLA-DR4, is affected in both. Like other autoimmune conditions, in PMR there is likely interplay between genetic and environmental factors causing a dysregulation of the immune system.

The European League Against Rheumatism (EULAR) / American College of Rheumatology (ACR) 2012 provisional classification criteria for PMR:
  • Patients 50 years or older
  • Bilateral shoulder pain not better explained by an alternative diagnosis
  • Presence of morning stiffness for more than 45 minutes
  • Elevated levels of C-reactive protein (CRP) and/or erythrocyte sedimentation rate (ESR)
  • New hip pain
In addition, the absence of peripheral (small joints of hands and feet) synovitis or of positive rheumatoid arthritis serology increases the likelihood of PMR.

Diagnosis of PMR is generally made on clinical grounds. The patient can present with slow subacute or chronic symptoms of malaise, fever, weight loss, night sweats, and anorexia. Pain and stiffness, rather than weakness, are common presenting symptoms.

Magnetic resonance imaging (MRI) and ultrasonography are equally effective in confirming PMR. Common shoulder lesions in PMR are subacromial or subdeltoid bursitis. Some patients present with "benign synovitis," which on ultrasound will not demonstrate true joint erosions. Glenohumeral joint synovitis and long-head biceps tenosynovitis can also coexist in PMR.

A patient's rapid response to corticosteroids may help confirm the diagnosis of PMR (steroids will decrease the pain associated with other inflammatory conditions as well).

PMR and GCA:
GCA is a systemic vasculitis affecting medium- to large-sized arteries, including the aorta and the extracranial branches of the carotid artery. PMR and GCA have a significant clinical association: 16% to 21% of cases of PMR are associated with GCA, and 40% to 60% of patients diagnosed with GCA also have PMR.

GCA is rarely found in individuals younger than 55. Patients may present with systemic symptoms such as new headache, ESR greater than 50 mm/hour, temporal artery tenderness to palpation, or decreased pulsation unrelated to arteriosclerosis. They may have visual disturbances, jaw claudication, or upper cranial palsies.
 
The most serious complication of GCA is blindness, a result of anterior ischemic optic neuropathy caused by ischemia secondary to inflammation of the ophthalmic artery supplying the optic nerve. All patients with suspected GCA should have temporal artery biopsy. Pathologic findings include a predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells.

Scalp necrosis is a rare but potentially life-threatening complication.

Codes

ICD10CM:
M35.3 – Polymyalgia rheumatica

SNOMEDCT:
65323003 – Polymyalgia rheumatica

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

PMR and GCA have a significant clinical association, and GCA should be suspected in patients with headache, visual disturbances, jaw claudication, upper cranial palsies, scalp tenderness, and/or decreased temporal artery pulse.

The differential diagnosis for PMR is broad and can be divided into inflammatory and noninflammatory, infectious, neoplastic, and of neuroendocrine origin. In each case, the history and physical examination along with other supporting evidence will aid in diagnosis. PMR involves the shoulders, neck, upper arms, thighs, and hips. The absence of shoulder involvement is rare and should prompt strong consideration of an alternative diagnosis.

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 05/09/2018
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Polymyalgia rheumatica : Neck pain, CRP elevated, ESR elevated
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