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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Please review our privacy policy. The steroids are usually administered as pulse IV therapy followed by oral steroids usually with subsequent taper [ 15syndroje3845 ]. However, their use may be considered with great care under special circumstances, for instance, intracardiac thrombi or massive pulmonary embolism. The V-Q scan in such patients shows area s with ventilation-perfusion mismatch. Support Center Support Center.

Hughes-Stovin syndrome.

Possible mechanisms to explain the prothrombotic nature of BD in literature include: All authors have read and approved the final manuscript. Neutrophils exhibit increased generation of reactive oxygen species ROSphagocytic capacity and cytokine production as well [ 536 ].

Management Owing to the lack of controlled trials, there are no standard treatment guidelines for the management of HSS. Close mobile search navigation Article navigation.

Similarly, the pathogenesis of pulmonary artery aneurysms in Hughes-Stovin syndrome has been attributed to weakening of the vessel wall due to inflammation, which corresponds well hughees the observation by Ketchum et al, that the aneurysms develop at the locations of prior thrombus and abnormal enhancement [ 9 ]. Pulmonary angiography showing an aneurysm of the interlobar pulmonary artery.

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Orphanet: Hughes Stovin syndrome

Macrophages containing hemosiderin may also be observed. Trauma for example, from a Swan-Ganz catheter 6. By using this site, you agree to the Terms of Use and Privacy Policy. A case of Behcet’s disease presenting with Hughes-Stovin syndrome. Open in a separate window.

Appropriate treatment, if instituted promptly and early in the huges of the disease, has the potential to induce remission [ 440475474 ].

He returned to America post-operatively for immunosuppressive treatment. In general there is a thrombus formation predisposition affecting the peripheral veins.

Overall, patients with HSS have a poor prognosis and aneurysmal rupture is the leading cause of death. Scleromalacia perforans in rheumatoid arthritis. For example, Herb et al have reported HSS in a patient who had an aneurysm of the left hepatic artery [ 21 ].

Unusual right ventricular thrombus in a woman with Hughes-Stovin syndrome. The first and second phases have to be present for the diagnosis of HSS while the third phase is the usual ultimate outcome for untreated patients.

Low pressure giant hugehs artery aneurysms in the adult: The management of HSS can either be medical or surgical. Syndrome of pulmonary artery aneurysms, pulmonary artery thrombi and peripheral venous thrombi.

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

Regardless the presence of deep venous thrombosis, the pulmonary artery thrombi may originate in situ, secondary to the pulmonary artery wall inflammation. Oxford University Press is a department of the University of Oxford. Also, it is not known whether patients in other reports on HSS were tested for HLA-B51 or not as no specific comment regarding such testing was made or alluded to in those reports.

Anti-HSV-1 antibodies observed more commonly in patients with Behcet’s disease than controls. Surgical resection provides an effective treatment option for patients with unilateral or localized PAAs and less invasive approaches such as transcatheter embolization may be feasible in some cases.

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Epidemiology HSS is an exceedingly rare disorder with less than 40 published cases in English medical literature. Arterial embolization is also an acceptable therapeutic option in patients with severe or recurrent hemoptysis [ 21 ]. J Thorac Cardiovasc Surg. Open in a separate window. In the absence of extensive thrombi, some authors have suggested the use of antiplatelet agents such as low dose aspirin in patients [ 6364 ].

Kim et al successfully used anticoagulation in a patient with HSS, hyperhomocysteinemia and intracardiac thrombi employing warfarin with enoxaparin as a bridge [ 45 ].

However other clinical presentations can overlap significantly; both are more common in young men, the pulmonary manifestations of both can be identical, syndroms the histology of the aneurysms syndroem both entities can be similar [ 8 ].

Diagnostic criteria and differential diagnosis of Behcets disease. Comparison with Conventional Angiography. Received Dec 15; Accepted Jan Large pulmonary artery aneurysm rupture in Hughes-Stovin syndrome: 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 stogin fluid cultures, more robust objective assessment is needed through the use of electron microscopy or 16 sRNA studies.