
Severe cardiovascular involvement in Hughes-Stovin syndrome
Author(s) -
Patrick GrogaBada,
Bernhard Klumpp,
Christian la Fougère,
Konstantin Nikolaou,
Christian Eick,
Meinrad Gawaz,
Theodoros Xenitidis
Publication year - 2017
Publication title -
case reports in internal medicine
Language(s) - English
Resource type - Journals
eISSN - 2332-7251
pISSN - 2332-7243
DOI - 10.5430/crim.v4n4p52
Subject(s) - medicine , etiology , vasculitis , thrombosis , thrombolysis , venous thrombosis , disease , cardiology , pathogenesis , vascular disease , surgery , myocardial infarction
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.