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Takayasu's arteritis has become increasingly recognized as a worldwide entity with a variable spectrum of disease expression. Although evidence for immune mechanisms of disease exist, the precise etiopathogenesis remains elusive. Improvements in the evaluation of disease activity beyond the current parameters of clinical symptoms, erythrocyte sedimentation rate, and angiographic findings are needed. Assessment of disease is handicapped by radiologic techniques that may detect diseased vessels but do not distinguish active from chronic lesions. A large proportion of patients with active disease respond to treatment with glucocorticoids, but a significant proportion will either require additional immunosuppression to prevent steroid toxicity or will remain unresponsive to treatment. At present, methotrexate appears to be an effective steroid-sparing agent in the treatment of active Takayasu's arteritis. Surgery continues to play a critical role in the long-term management of Takayasu's arteritis and is recommended when appropriate during inactive disease. Renal autograft transplantation has been offered as a new technique in the management of hypertension in Takayasu's arteritis.