Pulmonary artery hypertension as an initial manifestation of Takayasu's arteritis: A case report

Abstract

Takayasu's arteritis is a rare chronic vasculitis of unknown etiology. Symptomatic pulmonary artery disease may be the first sign of Takayasu's arteritis. We describe a 51-year-old woman who presented with severe dyspnea. The initial evaluation and routine exams suggested the presence of pulmonary hypertension of unknown etiology. Absence of bilateral radial and brachial pulses in the physical examination led to an angiographic study, which confirmed the diagnosis of Takayasu's arteritis with severe pulmonary hypertension. During bosentan therapy, the patient's clinical symptoms and exercise capacity improved and her 6-min walking distance increased. We emphasize the importance of considering Takayasu's arteritis in the early diagnosis and therapy of pulmonary artery hypertension.