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...levels. GCA symptoms develop abruptly in some patients but more often occur insidiously. Symptoms include headache, scalp tenderness, jaw claudication, and shoulder and hip pain and stiffness. Polymyalgia rheumatics occurs in up to half of patients with GCA. Some patients have aortic involvement. Information from both temporal artery biopsies and MRI studies can assist in making the diagnosis. High-dose corticosteroids and low-dose aspirin are the cornerstones of therapy, but corticosteroids have numerous adverse effects.
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Giant cell arteritis (GCA) is the most common form of primary systemic vasculitis. The disease preferentially affects the extracranial branches of the carotid arteries and, less often, causes clinical involvement of the aorta and its major branches.
GCA has an annual incidence of 20 cases per 100,000 persons older than 50 years; in the United States, the prevalence is estimated at 200 cases per 100,000 persons. (1) The disorder occurs almost exclusively in persons older than 50 years, exhibits 3:1 female-to-male predominance, and has a predilection for persons of northern European ancestry.
In patients with GCA, symptoms develop abruptly in a minority of patients but more typically occur in an insidious fashion and remain undiagnosed for weeks or months. The diagnosis is a clinical one, aided by information from temporal artery biopsies and, in some cases, MRI studies of the aorta and its primary branches. Classic histopathological findings include transmural inflammation with mononuclear cells and multinucleated giant cells. Laboratory investigation often demonstrates elevated acute phase reactant levels.
If not recognized promptly and managed appropriately, GCA may be associated with significant morbidity and mortality. High-dose corticosteroids are a cornerstone of therapy, but long-term management of this disorder may be challenging because of their numerous adverse effects. In this article, we describe a practical approach to making a diagnosis of GCA. We also offer guidelines for treatment with corticosteroids and other agents.
DIAGNOSIS
Clinical manifestations
The clinical presentation of GCA varies from patient to patient, but the classic symptoms and signs are well recognized. Most patients present with indolent symptoms that often are mistaken for infections or malignancies. The textbook symptoms include headache, scalp tenderness, jaw claudication, pain and stiffness in the shoulder and hip areas, weight loss, low-grade fever, and a general sense of being unwell.
In some cases-about 10%-the event that brings patients to medical attention is vision loss. This often occurs against a background of other symptoms that have gone unrecognized by practitioners or even by patients.
In some patients, cerebrovascular involvement caused by carotid or posterior circulation involvement results in strokes. However, true intracerebral vasculitis in GCA is extremely rare. Several atypical presentations are listed in Table 1, along with more characteristic presentations.
Headaches, often the first disease manifestation, vary in location and character. They may be dull, burning, or throbbing and have a range of severity from patient to patient. The main feature is that the headaches are new for that particular patient. Patients often...
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