Combined pulmonary vasodilator therapy and endovascular intervention for Hughes–Stovin syndrome

Abstract

Hughes–Stovin syndrome (HSS), a rare syndrome considered by some a form of Behçet's disease, is characterized by multiple pulmonary and/or bronchial artery aneurysms and thrombophlebitis [1,2]. It affects mainly young men, especially from Mediterranean countries and the Near East [2]. Acute manifestations include low-grade fever, weight loss, thrombophlebitis and hemoptysis [2]. The presence of pulmonary arterial aneurysms (PAA) carries a poor prognosis. Massive hemoptysis is the main cause of death.