Is Hughes-Stovin Syndrome a Particular Expression of Behçet's Disease?

François, M

doi:10.1378/chest.83.2.288a

Cited by 16 publications

(13 citation statements)

References 6 publications

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“…Some authors have suggested that HSS may actually be a partially manifested BD owing to their similar findings instead of a novel syndrome [16,19,38,39]. In fact, HSS and BD are the only vasculitides associated with the development of pulmonary artery aneurysms [4,40,41].…”

Section: Etiology and Pathogenesismentioning

confidence: 99%

Hughes-Stovin Syndrome

Khalid

Saleem

2011

Orphanet Journal of Rare Diseases

145

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Hughes-Stovin Syndrome (HSS) is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms. Less than 40 published cases of HSS have been described in English medical literature so far. The exact etiology and pathogenesis of HSS is unknown; possible causes include infections and angiodysplasia. HSS has also been considered as a variant of Behcet's disease (BD). Patients with HSS usually present with cough, dyspnea, fever, chest pain and haemoptysis. The management of HSS can either be medical or surgical. Medical management includes the use of steroids and cytotoxic agents. Cyclophosphamide, in particular, is a favored therapeutic agent in this regard. Antibiotics have no proven role in HSS while anticoagulants and thombolytic agents are generally contraindicated due to an increased risk of fatal hemorrhage. However, their use may be considered with great care under special circumstances, for instance, intracardiac thrombi or massive pulmonary embolism. For cases of massive hemoptysis due to large pulmonary aneurysms or those with lesions confined to one segment or one lung, lobectomy or pneumectomy can be carried out. However, surgical risks merit serious consideration and must be discussed with the patient. Transcatheter arterial embolization has emerged as a less invasive alternative to surgery in selected cases of HSS. Overall, patients with HSS have a poor prognosis and aneurysmal rupture is the leading cause of death. However, early diagnosis and timely intervention is crucial in improving the prognosis. There is a need to clearly elucidate the genetic, etiologic and pathologic basis for HSS in the future. Although most of the evidence put forward to refute the role of an infectious agent in the etiology and pathogenesis of HSS is based on negative blood and other body fluid cultures, more robust objective assessment is needed through the use of electron microscopy or 16 sRNA studies. The development of better therapeutic agents is also needed to address and prevent the serious consequences arising from pulmonary arterial aneurysms seen in BD and HSS. Also, the issue of anticoagulation in these patients is challenging and requires further deliberation.

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Section: Etiology and Pathogenesismentioning

confidence: 99%

Hughes-Stovin Syndrome

Khalid

Saleem

2011

Orphanet Journal of Rare Diseases

145

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“…There are similarities between Hughes-Stovin syndrome and Behçet's disease [7,8]. The angiographic and histological findings were comparable.…”

Section: Discussionmentioning

confidence: 66%

An unusual case of Hughes-Stovin syndrome

Herb¹,

Hetzel²,

Hetzel³

et al. 1998

Eur Respir J

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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.

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“…About 25% of patients with HSS develop thromboembolism, arterial aneurysms and vascular occlusions. The distribution of the vascular component of the syndrome is as follows: arterial (7%), venous (25%) or both (68%) [14]. Some authors have proposed that HSS may actually be a partially manifested Behçet's disease (BD) based on their similar findings [12,15,16].…”

Section: Discussionmentioning

confidence: 99%