Polymyalgia rheumatica is a syndrome closely associated with giant cell arteritis (temporal arteritis). It affects adults >
(See also Overview of Vasculitis.)
Polymyalgia rheumatica affects adults > 50; the female:male ratio is 2:1.
Because polymyalgia rheumatica is closely associated with giant cell arteritis, some authorities consider the 2 disorders to be different aspects of the same process. Polymyalgia rheumatica appears to be more common. A few patients with polymyalgia rheumatica develop giant cell arteritis, but 40 to 60% of patients with giant cell arteritis have polymyalgia rheumatica. Polymyalgia rheumatica may precede, follow, or occur simultaneously with giant cell arteritis.
Etiology and pathogenesis of polymyalgia rheumatica are unknown. Ultrasound and MRI findings suggest that there is low-grade axial synovitis and bursitis.
Symptoms and Signs of Polymyalgia Rheumatica
Polymyalgia rheumatica is characterized by bilateral proximal aching of the shoulder and hip girdle muscles and the back (upper and lower) and neck muscles. Stiffness in the morning is typical and lasts > 60 minutes. Shoulder symptoms reflect proximal bursitis (eg, subdeltoid, subacromial) and less often bicipital tenosynovitis or joint synovitis. Discomfort may awaken patients from sleep and is worse in the morning; occasionally it is severe enough to prevent patients from getting out of bed and from doing simple activities. The pain may make patients feel weak, but objective muscle weakness is not a feature of the disorder.
Diagnosis of Polymyalgia Rheumatica
Clinical findings
Exclusion of other causes
Polymyalgia rheumatica is suspected in older patients with typical symptoms, but other possible causes must be excluded.
Tests include erythrocyte sedimentation rate (ESR), C-reactive protein, complete blood count, thyroid-stimulating hormone levels, and creatine kinase (CK). In > 80% of patients, ESR is markedly elevated, often > 100 mm/hour, usually > 50 mm/hour (Westergren method). C-reactive protein is also elevated. Electromyography, biopsy, and other tests (eg, rheumatoid factor), which are normal in polymyalgia rheumatica, are sometimes done to exclude other clinically suspected diagnoses.
The following findings in polymyalgia rheumatica distinguish it from
Rheumatoid arthritis: In polymyalgia rheumatica, chronic small-joint synovitis, erosive or destructive lesions, rheumatoid nodules, and rheumatoid factor are absent in approximately 80% (although some joint swelling may be present) of patients. Differentiation from rheumatoid arthritis may be difficult in the remaining 20%.
Autoimmune myositis: In polymyalgia rheumatica, pain rather than weakness predominates; muscle enzyme levels and electromyography and muscle biopsy results are normal.
Hypothyroidism: In polymyalgia rheumatica, thyroid function test results and muscle enzyme levels are normal.
Multiple myeloma: In polymyalgia rheumatica, monoclonal gammopathy is absent.
Fibromyalgia: In polymyalgia rheumatica, symptoms are more localized, ESR is typically elevated, and pain is present with palpation and range of motion (active and passive) of the shoulders, even when the patient is distracted.
Treatment of Polymyalgia Rheumatica
Interleukin (IL)-6 inhibitor in selected patients
a diagnostic evaluation for giant cell arteritis should be done.
Treatment effectiveness is monitored by symptoms, ESR, and C-reactive protein. As symptoms subside, corticosteroids are tapered to the lowest clinically effective dose, regardless of ESR. C-reactive protein is more helpful than ESR in guiding response to treatment because ESR may be persistently elevated in older patients because of other reasons. Some patients are able to discontinue corticosteroids in approximately 2 years, or even sooner without relapse, whereas others require small doses for years. Nonsteroidal anti-inflammatory drugs (NSAIDs) are rarely sufficient.
Giant cell arteritis may develop at the onset of polymyalgia rheumatica or much later, sometimes even after patients appear cured of the disorder. Therefore, all patients should be instructed to immediately report headache, muscle pain during chewing, and, particularly, visual disturbances to their physician.
Treatment references
1. Spiera RF, Unizony S, Warrington KJ, et al. Sarilumab for Relapse of Polymyalgia Rheumatica during Glucocorticoid Taper. N Engl J Med 389(14):1263-1272, 2023. doi:10.1056/NEJMoa2303452
2. Caporali R, Cimmino MA, Ferraccioli G, et al. Prednisone plus methotrexate for polymyalgia rheumatica: a randomized, double-blind, placebo-controlled trial. Ann Intern Med 141(7):493-500, 2004. doi:10.7326/0003-4819-141-7-200410050-00005
Key Points
Polymyalgia rheumatica affects adults > 50 years, causing proximal myalgias and stiffness.
It is present in 40 to 60% of patients with giant cell arteritis.
Diagnose clinically, sometimes with supportive evidence of an elevated erythrocyte sedimentation rate and/or C-reactive protein along with a dramatic response to low to moderate doses of corticosteroids.
Treat with corticosteroids, eventually tapering if possible.
Warn patients about symptoms of giant cell arteritis.
