An unusual case of Hughes-Stovin syndrome

Abstract

In 1959 HUGHES and STOVIN described a syndrome consisting of multiple pulmonary aneurysms and peripheral venous thrombosis. Since that time several cases of the Hughes-Stovin syndrome have been published. We present the case of a 25 yr old man who, in addition to the classical symptoms, had multiple aneurysms of the bronchial arteries, which were the cause of severe haemoptysis. An aneurysm of the left hepatic artery was also detected. To our knowledge this is the first description of aneurysms of the bronchial arteries in Hughes-Stovin syndrome. Bleeding was successfully treated by double embolization of bronchial arteries via transfemoral catheter.

Síndrome Hughes-Stovin: Caso clínico

“…Los aneurismas observados pueden ser úni-cos, múltiples, unilaterales o bilaterales y no sólo pueden afectar la circulación pulmonar sino que también el resto de la circulación sistémica, como hepática e ilíacas, situación que tiene menos riesgo de rotura por las bajas presiones involucradas [11][12][13] .…”

“…La angiodisplasia de las arterias bronquiales también se ha propuesto como causa de los cambios vasculares del SHS, alterando la nutrición adecuada y dañando los vasavasorum, afectando el tejido elástico con la consiguiente formación de aneurismas 12 .…”

Thrombosis in a young woman: It’s not what you think

“…One patient described in the literature also had a hepatic artery aneurysm (Table 1). 4 One case report suggested hyperhomocys- teinemia may be involved in the formation of thrombi in HSS. 7 Hughes Stovin syndrome is considered to be a variant of Behcet's disease, 2,3 and these are the only two idiopathic syndromes known to predispose to the formation of pulmonary artery aneurysms.…”

Síndrome de Hughes-Stovin

“…The evidence is not very reassuring due to the fatal development of several cases, despite treatment. 4 Anticoagulation is controversial in the literature. Some authors question the real need of this treatment due to the essentially inflammatory etiology of thrombi, making them to be firmly adhered to the endothelium, minimizing the risks if emboli.…”

“…2 Hughes-Stovin syndrome is a rare condition, initially described in 1959, 3 is characterized by multiple pulmonary aneurysms and deep venous thrombosis. 4 Signs and symptoms include: cough, dyspnea, hemoptysis, headache, intermittent fever, papilledema, and those related with peripheral thrombophlebitis. 5 Rupture of an aneurysm inside the airways is the most common cause of morbimortality.…”