Takayasu's arteritis presenting as pulmonary hypertension

A. Haas and E. R. Stiehm

Takayasu's arteritis (pulseless disease) is a vasculitis that affects the large arteries, including the aorta, its branches, and the pulmonary arteries. A 15-year-old girl with Takayasu's arteritis was referred to our institution for marked pulmonary hypertension in the absence of symptoms referable to systemic vasculitis. She was shown to have a thickened, narrowed thoracic aorta, a stenosed right axillary artery, and severely affected pulmonary arteries. Despite corticosteroid therapy she died one month after diagnosis. This patient's illness indicates that pulmonary vessels might be the principal vessels involved in Takayasu's arteritis.