Polymyalgia rheumatica - Skin
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
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.
M35.3 – Polymyalgia rheumatica
65323003 – Polymyalgia rheumatica
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.
- Rheumatoid arthritis
- Fibromyalgia – diffuse pain present without clinical biomarkers
- Polymyositis – proximal weakness rather than pain, creatine kinase (CK) elevated
- Lumbar spinal stenosis – stiffness and pain restricted to the hip girdle region with or without MRI findings
- Statin-induced myositis – elevated CK, alleviation of symptoms after discontinuation of statin
- Parkinson disease – tremor, rigidity, bradykinesia, and coordination deficits of gradual onset
- Stigmata of endocarditis (see subacute bacterial endocarditis)
- Myelodysplastic syndrome
- Multiple myeloma
- Adhesive capsulitis / rotator cuff lesions
- Osteoarthritis – response to nonsteroidal anti-inflammatory drugs (NSAIDs), X-ray imaging showing narrowing of joint space and osteophytes
- Septic arthritis
- Hypothyroidism / hyperparathyroidism
- Amyotrophic lateral sclerosis – muscular weakness with ascending pattern without sensory involvement, with or without foot drop
- Polyarteritis nodosa