Hughes–Stovin syndrome is a rare autoimmune disorder of unknown cause that is characterized by the combination of multiple pulmonary artery aneurysms and . Learning points for clinicians. Pulmonary artery aneurysms (PAA) have a diverse differential diagnosis. Vasculitic features, without evidence of. Hughes-Stovin syndrome (HSS) is a rare autoimmune disorder, characterized by deep venous thrombosis (DVT) and pulmonary and/or bronchial aneurysms.

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Hughes-Stovin Syndrome: a case report and review of the literature

False aneurysm of the pulmonary artery with peripheral venous thrombosis. Anticoagulants and thombolytic agents are generally considered contraindicated due to stovim increased risk of fatal hemorrhage, even though they confer a beneficial effect in an embolic state [ 37 ]. Address correspondence to Dr C. Health care resources for this disease Expert centres Diagnostic tests 1 Patient organisations 19 Orphan drug s 0.

Trauma for example, from a Swan-Ganz catheter 6. Table 2 Autoantibodies proposed to be involved in pathogenesis of Behcet’s disease adapted from Mendoza-Pinto et al [ 5 ] and Kapsimali et al syndorme 36 ]. Antibiotics have no proven role in the management of HSS [ 122934 ].

Hughes–Stovin syndrome – Wikipedia

You can help by adding to it. Therefore, anticoagulation may be employed with great vigilance in a few carefully evaluated circumstances where the benefits are believed to significantly outweigh the risks. Aneurysmal rupture; it is the leading cause of death in patients of HSS. Furthermore, since aneurysms in HSS are usually hughez and multifocal at the time of diagnosis, embolization is a preferred modality in such patients [ 8 ]. Surgical resection provides an effective treatment option for patients with hughess or localized PAAs and less invasive approaches such as transcatheter embolization may be feasible in some cases.


N Engl J Med. It is speculated that HLA-B51 forms an integral part of an immunologic axis in patients with BD that interacts with cross-reacting self-antigens and immunoglobulin-like receptors on immune stovvin of the body [ 36 ].

Eur J Cardiothorac Surg. Etiopathogenesis of Behcet’s disease.

Hughes-Stovin syndrome.

There is a need to clearly elucidate the genetic, etiologic and pathologic basis for HSS in the future. Antiplatelet agents In the absence of extensive thrombi, some authors have suggested the use of antiplatelet agents such hhughes low dose aspirin in patients [ 6364 ]. Most of the data on HSS is in the form of synxrome case reports.

Anticoagulation may prevent the progression of pulmonary embolism and resolve vein thrombi, but since it increases the risk and severity of haemoptysis, it is contraindicated. Acknowledgements We are greatly indebted to the reviewers for their valuable suggestions and critique.

Nihon Kyobu Shikkan Gakkai Zasshi. Autoantibody to Retinal S antigen 5.

They first described the findings of the syndrome deep venous thrombosis and segmental pulmonary artery aneurysms in a total of four syndrone patients with pulmonary artery aneurysms in [ 1 ]. Antineutrophilic cytoplasmic antibody ANCA 3. 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.


A copy of the written consent is available for review by the Editor-in-Chief of this journal. Surgical management For the cases of massive hemoptysis due to large pulmonary aneurysms or those hguhes lesions confined to one segment or one lung, lobectomy or pneumectomy can be carried out to remove the aneurysms based on the data from published case reports. The natural course of the illness is usually fatal because of fulminant haemoptysis.

Orphanet: Hughes Stovin syndrome

However, it is only safe to perform this procedure in the early stages [ 72 ]. Macrophages containing hemosiderin may also be observed. Rarely, the patients may require repeat embolization syncrome the arterial lesions may become recanalized or revascularized [ 21 ]. Epidemiology HSS is an exceedingly rare disorder with less than 40 published cases in English medical literature.

However, the caveat here is that patients may still develop thrombosis despite adequate anticoagulation [ 38 ]. Antibiotics have no proven role in HSS while anticoagulants and thombolytic agents are generally contraindicated due to an increased sfovin of fatal hemorrhage.