Polymyalgia Rheumatica and Giant Cell Arteritis BRIAN UNWIN, COL, MC, USA, CYNTHIA M. WILLIAMS, CAPT (R), MC, USN, and WILLIAM GILLILAND, COL, MC, USA, Uniformed Services University of the Health Sciences, Bethesda, Maryland Polymyalgia rheumatica and giant cell arteritis are common, closely related vasculitic conditions that almost exclusively occur in patients older than 50 years. They may be manifestations of the same underlying disease and often coexist. Patients with polymyalgia rheumatica usually present with acute onset of stiffness and pain in the shoulder and pelvic musculature, which may be accompanied by fever, malaise, and weight loss. If untreated, polymyalgia rheumatica may result in significant disability. Giant cell arteritis may manifest as visual loss or diplopia, abnormalities of the temporal artery such as tenderness or decreased pulsation, jaw claudication, and new-onset headaches. Erythrocyte sedimenta- tion rate and temporal artery biopsy help make the diagnosis. Giant cell arteritis requires urgent diagnosis because without treatment it may lead to irreversible blindness. Patients with either condition also may have nonspecific symptoms. Corticosteroids are the mainstay of therapy for both conditions, with higher doses required for treatment of giant cell arteritis. Duration of corticosteroid therapy can be five years or longer before complete clinical remission is achieved. Monitoring for corticosteroid-associated side effects such as osteoporosis and diabetes, as well as for relapses and flare-ups, is key to chronic management. The prognosis for either condition, if treated, is good. (Am Fam Physician 2006;74:1547-54, 1557-8. Copyright © 2006 American Academy of Family Physicians.) ◆ Patient information: olymyalgia rheumatica (PMR) and GCA is less common, with an annual inci- A handout on polymyalgia giant cell arteritis (GCA; also known dence in persons older than 50 of approxi- rheumatica and giant cell arteritis, written by Jill as temporal arteritis) are common, mately 18 per 100,000. In both conditions the Giordano, MSIV, University interrelated inflammatory disorders incidence peaks in those between 70 and 80 of Medicine and Dentistry Pthat occur predominantly in persons older years of age, and diagnosis is more common of New Jersey School of than 50 years. GCA most commonly involves in northern latitudes.6 Osteopathic Medicine, is provided on page 1557. the temporal artery, but arteries in other parts of the body also can be inflamed. It is Pathogenesis the most common primary vasculitis among Although the pathogeneses of GCA and older persons and can lead to blindness if not PMR are uncertain, similar cellular immune diagnosed and treated in a timely manner.1 responses involving T cells, antigen-present- About 50 percent of persons with GCA also ing cells, macrophage-derived inflammatory have PMR, and about 10 percent of those cytokines, genetic human leukocyte antigen with PMR also have GCA.2 PMR manifests as molecules, and macrophages are found in severe stiffness and pain in the girdle muscles both conditions.7 Because there is so much (i.e., neck, shoulders, buttocks, and thighs); clinical and pathophysiologic overlap, the the forearms, hands, calves, and feet usually conditions are thought to be manifestations are not affected.3 Without treatment, PMR of the same disease. can cause significant disability. In GCA, a syndrome of systemic inflam- mation accompanies the vascular mani- Epidemiology festations. Arterial biopsies often reveal The most important risk factor for both inflammatory changes to the tunica media conditions is older age, and the number of vasorum and tunica adventitia, which cause persons at risk in developed countries is narrowing or occlusion of the vessel lead- expected to double in the next 25 years as ing to ischemia distal to the lesion. GCA the average age of the population increases.4 most commonly affects the branches of PMR has a prevalence of one in 133 among the internal and external carotid arteries. persons older than 50, with women two The involvement of these branches leads times more likely to be affected than men.5 to the clinical findings of headache, jaw Downloaded from the American Family Physician Web site at www.aafp.org/afp. Copyright © 2006 American Academy of Family Physicians. For the private, noncommercial use of one individual user of the Web site. All other rights reserved. Contact [email protected] for copyright questions and/or permission requests. PMR and GCA SORT: KEY Recommendations FOR PRACTICE Evidence Clinical recommendation rating References If the erythrocyte sedimentation rate is normal, then GCA is unlikely. C 11 Patients older than 50 years with jaw claudication; diplopia; and C 11, 14 temporal artery beading, prominence, and tenderness should have a temporal artery biopsy to diagnose GCA. Prednisone should be given as first-line therapy for treatment of B 11, 14 PMR and GCA. Treatment of GCA should not be delayed while awaiting biopsy. C 14 Ultrasonography and positron emission tomography are not B 18-20 replacements for temporal artery biopsy. GCA = giant cell arteritis; PMR = polymyalgia rheumatica. A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evi- dence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, see page 1463 or http://www.aafp.org/afpsort.xml. claudication, scalp tenderness, and blind- thighs; low-grade temperature elevation; ness.7 However, GCA can affect vessels any- evidence of weight loss; and synovitis. Distal where in the body.4,8,9 extremity manifestations are more promi- In PMR, the systemic inflammatory nent in PMR than in GCA. These findings response is the most prominent feature, but may include asymmetrical peripheral wrist inflammation of the blood vessels remains and knee arthritis, carpal tunnel syndrome, clinically undetectable. and swelling of the hands and feet.10 Diagnosis GIANT CELL ARTERITIS Distinguishing between GCA and PMR is GCA often manifests as a new-onset headache important because GCA can lead to blindness or a headache that is different from previous and requires higher doses of medication. headaches. The headache usually is ongo- ing for two to three months before patients POLYMYALGIA RHEUMATICA seek medical attention. Other common The onset of PMR usually is acute. However, symptoms generally are present for longer than one month before patients seek an eval- TABLE 1 uation. Table 14,6 summarizes the findings Findings Associated with associated with a diagnosis of PMR. This dis- Polymyalgia Rheumatica ease can be functionally devastating to older adults. Shoulder pain is the most common Age 50 years or older symptom,6 and the pain and stiffness experi- Erythrocyte sedimentation rate greater than enced in the shoulders and upper arms may 50 mm per hour make hygiene and self-care tasks difficult. Mild, normochromic, normocytic anemia The physical examination of a patient Aching, pain, and morning stiffness in the with PMR often is less striking than the shoulders and upper arms, hips and thighs, history would suggest; however, findings or neck and torso may include limited range of motion in the Symptoms of systemic inflammation (e.g., anorexia, depression, fever, malaise, night neck, shoulders, and hips secondary to pain sweats, weight loss) in the associated proximal muscles; inflam- mation of the bursae in the shoulder and hip Information from references 4 and 6. regions; tenderness of the upper arms and 1548 American Family Physician www.aafp.org/afp Volume 74, Number 9 ◆ November 1, 2006 PMR and GCA reveal reduced range of motion in the shoul- TABLE 2 der and hip because of pain, or a more distal Findings Associated with synovitis, usually of the wrists.11 Giant Cell Arteritis Temporal artery biopsy should be per- formed when clinical and laboratory evi- Age 50 years or older dence suggests GCA.14 One meta-analysis Erythrocyte sedimentation rate greater than found only a few features were predictive 50 mm per hour of a positive temporal artery biopsy.11 Find- Anemia ings in GCA that increase the likelihood of Headache: temporal with temporal artery a positive temporal artery biopsy result are involvement, or occipital with occipital artery 11 involvement listed in Table 3. A normal erythrocyte Jaw claudication with variant presentation: sedimentation rate generally excludes the usually occurs when chewing tough foods; diagnoses of GCA and PMR. vague discomfort around the jaw; diffuse mandibular discomfort; dental discomfort; DIFFERENTIAL DIAGNOSIS sinus pain and pressure; tongue pain The differential diagnosis for GCA and PMR Abnormal temporal artery includes diseases and conditions that may Constitutional symptoms: fever, weight loss, present with pain, fatigue, weight loss, and depression, malaise fever. The most common conditions that Polymyalgia rheumatica should be considered are listed in Table 4.15 Visual symptoms: partially obscured vision, blindness from occlusion of posterior ciliary In a review, about 16 percent of patients with artery, amaurosis fugax, diplopia, visual fever of unknown origin were diagnosed with field cuts GCA.16 Infectious diseases, such as viral infec- Arthralgias tions, endocarditis, hepatitis (A, B, and C), and human immu- Information from references 4, 6, 11, and 12. nodeficiency virus or acquired The erythrocyte sedimen- immunodeficiency syndrome, tation rate is the most also should