Severe cardiovascular involvement in Hughes-Stovin syndrome

Abstract

Hughes-Stovin syndrome (HSS) is a rare autoimmune vasculitis and is characterized by the simultaneous presence of deep venous thrombosis and pulmonary artery aneurysms. The exact etiology and pathogenesis of this life-threatening syndrome is currently unknown. The disease is thought to be a variant of Behcet’s disease with major vascular involvement. Here we report a case of a 19-year-old man from West Africa with a severe cardiovascular manifestation of HSS. The patient was referred to our hospital with dyspnoea, recurrent fever and swelling of the left leg. Echocardiography revealed extensive biventricular thrombi. He responded very well to immunosuppressive therapy in combination with anticoagulation using low-molecular-weight heparins (LMWHs). Thrombolysis was consciously avoided.