GIANT CELL ARTERITIS AND POLYMYALGIA RHEUMATICA

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DEFINITIONS

Giant cell arteritis31 is also referred to as temporal arteritis,44 cranial arteritis,51 and granulomatous arteritis .63 The disease occurs in persons over 50 years of age, and 90% are over age 60 at onset. The cranial branches of the arteries originating from the arch of the aorta are involved most prominently, but the arteritis may be more generalized.

Polymyalgia rheumatica6 is characterized by aching and morning stiffness in the proximal portions of the extremities and torso. Definitions

INCIDENCE

Giant cell arteritis is relatively common in central and northern Europe and in the United States. Average annual incidence rates have been as high as 27 per 100,000 persons aged 50 years and older.4, 7, 28, 50, 60, 65, 66, 79, 82 In the population of Olmsted County, Minnesota, 125 cases were identified over a 42-year period,79 which formed an average annual incidence rate of 17.8 cases per 100,000 population aged 50 and older and a prevalence of persons with a history of giant cell arteritis

CAUSE AND PATHOGENESIS

Investigations of polymyalgia rheumatica and giant cell arteritis in the past several years have improved understanding of these conditions.48 Although the increasing incidence of giant cell arteritis and polymyalgia rheumatica after the age of 50 years implies a relationship to aging, the specific factors underlying this observation are not understood. Reports of familial aggregation and the high incidence rates in northern Europe and in persons in the United States with similar ethnic

PATHOLOGY

Arteries that originate from the arch of the aorta are affected to the greatest degree, but almost any artery of the body may be affected occasionally as well as some veins.36, 52, 55 The arteries tend to be affected in a segmental or patchy fashion, but long portions of arteries may be involved. In patients who died during the active phase of giant cell arteritis, the highest incidence of severe involvement has been noted in the superficial temporal arteries, vertebral arteries, and ophthalmic

CLINICAL FEATURES

The mean age at onset of giant cell arteritis and polymyalgia rheumatica is approximately 70 years, with a range of about 50 to more than 90. Women are affected about twice as commonly as men.29, 78, 79 The onset may be abrupt, but in most instances the symptoms have been present for weeks or months before the diagnosis is established. Constitutional symptoms, including fatigue, anorexia, and weight loss, are present in the majority of patients and may be an early or even an initial finding.

RELATIONSHIP BETWEEN POLYMYALGIA RHEUMATICA AND GIANT CELL ARTERITIS

Alestig and Barr3 were early investigators who reported finding temporal arteritis in patients with polymyalgia rheumatica who had no direct symptoms or signs of vasculitis. In several reports of giant cell arteritis, polymyalgia rheumatica has been noted in about 40% to 60% of patients and has been the initial symptom complex in 20% to 40%.12, 25, 40, 47, 66 Conversely, in series of patients with polymyalgia rheumatica, giant cell arteritis has been found in 0 to 80% of cases.12, 47 The cause

LABORATORY STUDIES

Hematologic test results in polymyalgia rheumatica and giant cell arteritis are similar.12 A mild-to-moderate normochromic anemia is usually present in both during their active phases. Leukocyte and differential counts are generally normal. A markedly elevated erythrocyte sedimentation rate is characteristic of both. Levels of greater than 100 mm in 1 hour (Westergren method) are common, but occasionally untreated biopsy-proven cases of giant cell arteritis or polymyalgia rheumatica may have

DIAGNOSIS

Giant cell arteritis should be considered in the diagnosis of any patient older than 50 years of age who has a new form of headache, abrupt loss of vision, polymyalgia rheumatica, unexplained prolonged fever or anemia, and high erythrocyte sedimentation rate. The manifestations vary in severity and may be transient, so patients suspected of having this disease must be questioned carefully about recent as well as current symptoms. The arteries of the head, neck, upper torso, and arms should be

TREATMENT AND COURSE

Once the diagnosis of giant cell arteritis is established, glucocorticoid treatment should be instituted. In patients strongly suspected of having giant cell arteritis, especially those with recent or impending vascular complications such as visual loss, therapy may be started immediately or after a hemoglobin and erythrocyte sedimentation rate are obtained. Steroid treatment alters the histopathologic picture, but inflammatory changes appear to be recognizable for 2 to 4 weeks or more after

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    Address reprint requests to Gene G. Hunder, MD, Mayo Foundation, 200 First Street Southwest, Rochester, MN 55905

    *

    From the Division of Rheumatology, Mayo Clinic, and the Department of Medicine, Mayo Medical School, Mayo Foundation, Rochester, Minnesota

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