Recognition and Management of Polymyalgia Rheumatica and Giant Cell Arteritis
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Am Fam Physician. 2013 Nov 15;88(10):676-684.
Patient information: See related handout on polymyalgia rheumatica and giant cell arteritis, written by the authors of this article.
Author disclosure: No relevant financial affiliations.
Polymyalgia rheumatica affects proximal muscles and joints, causing disability in older adults. Giant cell arteritis affects medium and large arteries and can result in blindness. These conditions overlap significantly, often occurring together. Despite the similarities, each has distinct symptoms, corticosteroid dosing requirements, and prognosis. The hallmark of both conditions is inflammation. Polymyalgia rheumatica primarily affects the shoulders, neck, and hips with prominent bilateral pain. Systemic findings such as fatigue and weight loss are common, and there is no definitive diagnostic test. Moderate-dose corticosteroid therapy with a slow taper rapidly resolves symptoms. Management of patients responding to treatment can occur in the primary care setting, if there is no concomitant giant cell arteritis. The clinical presentation of giant cell arteritis varies widely, from new-onset headache and constitutional symptoms, to jaw claudication, to less common isolated visual changes and upper extremity claudication. Treatment requires higher dosages of corticosteroids and urgent referral to a rheumatologist. Relapse is common in both diseases. Surveillance is important, as is monitoring for long-term complications of corticosteroid use. Osteoporosis management and gastrointestinal ulcer prophylaxis should be initiated. The primary care physician's coordination of care with a rheumatologist and with other subspecialists, if needed, is essential in the management of giant cell arteritis.
Polymyalgia rheumatica, the most common chronic inflammatory condition in older adults, primarily affects proximal muscles and joints, causing disability. Giant cell arteritis, the most common type of vasculitis in adults, affects medium and large arteries and can result in blindness.1,2 These conditions often occur together and are among the most common diseases that necessitate long-term corticosteroid use in older adults.3,4 They are often identified and treated, at least in part, by the primary care physician. Polymyalgia rheumatica is two to three times more common than giant cell arteritis,3 but there is significant overlap between the two conditions. Approximately 18% to 26%3,5 of cases of polymyalgia rheumatica involve giant cell arteritis; conversely, 27% to 53%6,7 of cases of giant cell arteritis involve polymyalgia rheumatica. Polymyalgia rheumatica can occur before, with, or after the emergence of giant cell arteritis.8
Both conditions affect similar populations (typically older white persons of Northern European descent).5 The prevalence varies by age and population. In areas with higher concentrations of persons of Northern European descent and in persons older than 50 years, the prevalence is one in 143 persons for polymyalgia rheumatica and one in 500 for giant cell arteritis.9 The incidence of both conditions increases with age, peaking between 70 and 80 years of age.3 Women are two to three times more likely than men to be affected by either condition.1,5 Blacks, Asians, and Alaska natives have a much lower incidence of these diseases.3,10,11 Despite their similarities, polymyalgia rheumatica and giant cell arteritis have distinct symptoms, corticosteroid requirements, and prognoses. When the conditions occur together, the higher corticosteroid doses used to treat giant cell arteritis are needed to prevent significant complications.12
The hallmark of these conditions is inflammation.3,13 The etiologies are not clearly defined. Environmental exposures and genetics both play a role, with antigen-mediated inflammation the likely culprit.3,13 Seasonal, cyclical variations implicate infectious agents (e.g., viruses, Mycoplasma pneumoniae) as triggers, although specific pathogens have not yet been identified.13 Until recently, evidence of actual muscle inflammation was lacking in polymyalgia rheumatica, given normal muscle enzymes and inconsistent immunohistologic findings or vasculitis.14,15 Traditionally, inflammation was thought to be limited to the synovial and bursal linings.14,16 However, inflammatory cytokines have now been found in symptomatic muscles, indicating muscle involvement.17 In giant cell arteritis, inflammation affects medium and large arteries.1 Histologically, focal and segmental lesions are characterized by granulomatous, inflammatory infiltrates at the intimamedia junction that form skip lesions.18
SORT: KEY RECOMMENDATIONS FOR PRACTICE
|Clinical recommendation||Evidence rating||References||Comments|
Straightforward polymyalgia rheumatica should be treated with low-dose corticosteroids and a slow taper.
Prednisone in a starting dosage of 15 mg per day is the consensus recommendation.
Medications should be instituted to decrease fracture risk in patients on high-dose, long-term glucocorticoid therapy.
Alendronate (Fosamax) and risedronate (Actonel) have been shown to increase bone mineral density and decrease vertebral fractures in patients on long-term glucocorticoid
TONYA L. CAYLOR, MD, is a faculty member at the Alaska Family Medicine Residency in Anchorage. She is also a clinical assistant professor in the Department of Family Medicine at the University of Washington School of Medicine in Seattle.
ALLEN PERKINS, MD, MPH, is a professor in and chair of the Department of Family Medicine at the University of South Alabama in Mobile.
Address correspondence to Allen Perkins, MD, MPH, University of South Alabama, 1504 Springhill Ave., Ste. 3414, Mobile, AL 36604. Reprints are not available from the authors.
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