The clinical spectrum of Takayasu's arteritis

Takayasu's arteritis is a rare inflammatory disease of the aorta, its major branches, and the pulmonary artery in which the varying anatomic involvement, the time course, and the periodicity of exacerbations give rise to a wide variety of signs and symptoms. We have recently encountered five patients with Takayasu's arteritis whose symptoms and findings demonstrate the clinical spectrum of this disease. All five patients are women, with a current mean age of 41 years. Although findings at initial evaluations included systemic manifestations and elevation of the erythrocyte sedimentation rate in four patients, the fifth patient had a normal erythrocyte sedimentation rate and signs of abdominal aortic occlusion. Two patients had a history of hypertension, and four patients complained of upper- or lower-extremity claudication. Arteriographic examination revealed aortic arch branch vessel involvement (type I) in two patients who also had aortic valvular insufficiency; three patients had combined arch vessel and distal aortic disease (type III). All patients have been maintained on steroid medications, and one patient has undergone a trial of cytotoxic agents. Three patients underwent surgical procedures: aortic valve replacement in two patients, and aortorenal bypass in one patient. Takayasu's arteritis gives rise to a variety of symptoms and findings resulting from the distribution and severity of the inflammatory process. With adequate immunosuppression and selective application of surgical therapy, there is a good prognosis for survival and a return to functional status.