Giant cell arteritis, also known as temporal arteritis, cranial arteritis, and granulomatous arteritis, is a vasculitis of undetermined origin. Even though the histological findings vary, the characteristic features include lymphocytic infiltration with fragmentation of the internal elastic lamina, granulomatous inflammation, histiocytes, and multinucleated giant cells. These classic histological features are seen in approximately 60 per cent of temporal artery biopsies. Giant cell arteritis is responsible for 26 per cent of all primary vasculitic syndromes in North America. The disease can occur in association with polymyalgia rheumatica, and affects middle-aged or older individuals. There are no specific laboratory tests to diagnose giant cell arteritis, although a majority of patients exhibit an elevated erythrocyte sedimentation rate. Biopsy of the temporal artery is necessary to document the diagnosis.
Pulmonary complications of giant cell arteritis occur in approximately 10 per cent of patients. The respiratory symptoms can be the presenting features, and they include cough, sore throat, and hoarseness. A population-based study of 94 patients with giant cell arteritis found pulmonary symptoms in up to 30 per cent. It should be considered in any older patient with a new cough or throat pain without obvious cause. Disease of the small airways has been observed in 46 per cent of patients with giant cell arteritis; however, the abnormalities have not been significant. Chest radiograph abnormalities consist of pulmonary nodules, interstitial infiltrates, and occlusion and aneurysms of the pulmonary artery.
Virtually all manifestations of the disease respond favorably to systemic corticosteroids at an initial dose of 1 mg/kg/day. Chronic low-dose therapy is required to maintain remission in most patients.
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