Hughes-Stovin syndrome, a rare cause of thrombosis and pulmonary artery aneurysm: a case report

Abstract

Hughes-Stoven syndrome (HSS) is a rare disorder with characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysm that has been considered a variant of Behcet Disease (BD). A case report will be discussed which initially thought to be either BD or pulmonary embolism. A male, 30 years old patient, was under pneumonia treatment in another clinic due to dypsnea, phlegma, coughing, fever and night sweating. After a month after treatment, he had hemoptysis, starting from minimal bleeding upon coughing to 300 cc daily. Initial evaluation with computed tomography angiography (CTA) revealed filling defects in favor of embolism at segments of bilateral pulmonary arteries and pulmonary artery aneurysm. To evaluate aneurysm, arterial phase CTA was used and a few aneurysms up to 12 mm with thromboembolism within were seen in both pulmonary arteries. Further investigation revealed presence of bilateral lower extremity deep vein thrombosis, increased sedimantation and c-reactive protein levels and a normal echocardiography. Originally thought to be BD, due to young age, oral lesions and aneurysms, diagnosis was accepted as HSS. Medical management includes use of steroid and cytotoxic agents. Anticoagulants and thrombolytic agents are contraindicated. Our patient was treated by steroid and cyclophosphamide for six months however patient did not radiologically improved as expected and treatment was extended for 3 more months. Currently patient is stable and can continue his daily life. Presence of hemoptysis, pulmonary aneursyms, thrombosis should arise the suspicion of HSS, especially in younger patients, despite the rarity of the disease due to its possible mortal complications.