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 years old man who presented with inferior vena caval thrombosis with recurrent hemoptysis. There were evidences of pulmonary artery aneurysm and thrombosis of Inferior Vena Cava (IVC) and lower extremity veins on Contrast Enhanced CT (CECT) scan of the thorax and color doppler study respectively. There were no orogenital ulcerations or uveitis consistent with Behçet's disease. CT angiography was planned but the patient died following massive hemoptysis probably due to rupture of the aneurysm.