Abstract

Hughes–Stovin syndrome (HSS) is a very rare clinical entity, which is characterized by peripheral venous thrombosis and multiple pulmonary and/or bronchial artery aneurysms and is associated with a high mortality. Although the exact etiology of HSS is still not known, possible causes include infection and angiodysplasia. Because of its histopathological and clinical similarities with Behçet's disease (BD), it is also considered as a variant of BD. HSS usually present with cough, dyspnea, fever, chest pain, and hemoptysis, which can be massive and often fatal. We report a case of a 24-year-old man who presented with thrombosis of inferior vena cava (IVC) with recurrent hemoptysis. There were evidences of pulmonary artery aneurysm and thrombosis of IVC and lower extremity veins on contrast-enhanced computed tomography (CECT) scan of the thorax and color Doppler study, respectively. There were no orogenital ulcerations or uveitis consistent with BD. Pulmonary transcatheter embolization was planned but the patient died following massive hemoptysis probably because of rupture of the aneurysm.

Key words: Hughes–Stovin syndrome, pulmonary artery aneurysm, hemoptysis