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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Pulmonary manifestations of Behcet’s disease. Multiple, small PAA warrant early immunosuppression.

This is usually achieved by starting the patient on intravenous or subcutaneous heparin and then shifting to oral warfarin therapy. Other agents that have been variably sgndrome in the treatment of HSS include colcichine, cyclosporine and azathioprine syncrome 81538 ].

False aneurysm of the pulmonary artery with peripheral venous thrombosis. Some authors have suggested that HSS may actually be a partially manifested BD owing to their similar findings instead of a novel syndrome [ 16193839 ].

However, the caveat here is that patients may still develop thrombosis despite adequate anticoagulation [ 38 ]. Check this box if you wish to receive a hughws of your message. Support Center Support Center. The exact etiology and pathogenesis of HSS is unknown; possible causes include infections and angiodysplasia.

Segmental pulmonary artery aneurysms with peripheral venous thrombosis. Active vasculitis that can lead to thrombosis [ 73 ]. Autoimmune diseases Syndromes Disease stubs Cardiovascular system stubs.

Orphanet: Hughes Stovin syndrome

Conventional angiography can be used for better evaluation of the pulmonary aneurysms and assessment of angiodysplastic bronchial arteries in Hughes-Stovin syndrome. For patients who are not suitable candidates for aggressive surgical intervention, transcatheter arterial embolization, being a less invasive procedure, offers a suitable and effective alternative in HSS [ 8 ].

Heat shock proteins – Role for heat shock proteins of mycobacteria and streptococci suggested in Behcet’s disease. Recurrent phlebitis also commonly involves the large vessels, resulting in thrombus formation.


Multiple pulmonary artery aneurysms. As with any genetic disease, knowledge of the latter will be helpful to clinicians in the provision of pre-conception genetic counseling to patients with HSS. Management and treatment Initial management of HSS often involves administration of corticosteroids, usually in combination with cytotoxic agents intravenous cyclophosphamide followed by oral azathioprine to stabilize the PAAs.

Hughes-Stovin Syndrome

Cyclosporine A, being neurotoxic, should not be used in patients with neurological manifestations of BD [ 58 ]. CT scan of the chest showing ill defined infiltrate in lower lobe of the right lung. Kindermann et al have described the resection of pulmonary artery aneurysm with the reconstruction of the arterial segment using a saphenous vein grafts [ 66 ].

According to the initial hypothesis [ 1 ], pulmonary artery aneurysms may develop from a degenerative defect in the bronchial arteries or may even be mycotic in origin resulting from emboli infected with low-grade virulence organisms.

For BD, the following infectious agents have been implicated in the pathogenesis but never conclusively proven: Specifically, pulmonary involvement is often indistinguishable between the two entities.

Authors have performed embolization with several agents including steel coils, ethibloc and an epoxy, isobutyl cyanoacrylate [ 8 ]. Chlamydia pneumoniae – IgG seropositivity for C.

In the pulmonary veins, fibrosis and muscular medial thickening is seen. In fact it is reported that multi-detector row helical CT angiography provides more precise depiction of bronchial and nonbronchial systemic arteries than does conventional angiography [ 14 ]. TS conceived the project, performed the literature search, interpreted the data, drafted the manuscript and critically revised it. Report of a case in a year-old boy and a review of the literature.

Most commonly, immunosuppressive therapy involving a combination regimen of glucocorticoids and cyclophosphamide has been employed as a first line medical management in the treatment of HSS, although its effectiveness remains to be fully established [ 840 ].


British Journal of Diseases of the Chest. J Vasc Interv Radiol. Although it should be acknowledged that HSS and BD share many clinical, radiological and histological features, the suggestion that they are, in essence, the same disease can neither be currently accepted nor categorically refuted because the pathologic, genetic and etiologic basis of both conditions has not been clearly unraveled.

Doppler ultrasound of extremities Peripheral venous thrombosis is an important part of HSS. They performed manuscript writing and revision. Pulmonary arterial thrombo-embolism and pulmonary arterial mycotic aneurysms in cattle with vena caval thrombosis: Authors’ contributions UK performed the literature search, interpreted the data and drafted the manuscript. Etiology The etiology of HSS is unknown; however, it is assumed that HSS is a form of vasculitis following a similar mechanism of pathogenesis to that thought to be involved in BD.

However, early diagnosis and timely intervention is crucial in improving the prognosis. Magnetic resonance angiography MRA is relatively less sensitive than helical CT for picking up small aneurysms [ 20264749 ].

Pathogenesis, imaging investigation and treatment of this syndrome are also briefly discussed.

We are greatly indebted stogin the reviewers for their valuable suggestions and critique. SCVIR annual meeting film panel session: All authors have given final approval of the version to be published.

Rare case of multiple pulmonary artery aneurysms with caval thrombosis–Hughes-Stovin syndrome. A timely diagnosis and intervention is imperative to prevent potentially life-threatening massive hemorrhage due to pulmonary aneurysms [ 12 ].

Antineutrophilic cytoplasmic antibody ANCA 3. The material is in no way intended to replace syndroem medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.