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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Table 2 Autoantibodies proposed to be involved in pathogenesis of Behcet’s disease adapted from Mendoza-Pinto et al [ 5 ] and Kapsimali et al [ 36 ]. You can help by adding to it.

Orphanet: Hughes Stovin syndrome

J Magn Reson Imaging. Thus, the genetic basis and familial predisposition of HSS remains nebulous.

However, the high morbidity and mortality associated with surgery, and the frequent bilaterality and multifocality of the pulmonary artery aneurysms at the time of diagnosis, makes transcatheter embolization an alternative to surgery in most cases [ 2 ]. It also aids in assessment of angiodysplastic bronchial arteries in HSS. However, immunologic mechanisms, different from those in other autoimmune diseases, are believed to be involved in the pathogenesis of Huvhes.

By that stoviin, long term anticoagulation may have the potential to prevent further aneurysm formation by targeting this particular mechanism [ 62 ]. For BD, the following infectious agents have been implicated in the pathogenesis but never conclusively proven: He returned to America post-operatively for immunosuppressive treatment.

Laboratory findings The laboratory findings in HSS patients are non-specific. Massive hemoptysis and deep venous thrombosis presenting in a woman with Hughes-Stovin syndrome: It usually affects the young adult population bracket reported cases ranged in age from 12 to 48 years and holds a strong predilection for the male gender [ 8 – 10 ].

Similarly Mahlo et al. However, surgical risks merit serious consideration and must be discussed with the patient. In the absence of extensive thrombi, some authors have suggested the use of antiplatelet agents sgovin as low syndrom aspirin in patients [ 6364 ].


Hughes-Stovin Syndrome

N Engl J Med. Possible mechanisms to explain the prothrombotic nature of BD in literature include: HSS huhhes been variably described as “the cardiovascular manifestation of Behcet’s disease” [ 14 ], “incomplete Behcet’s” [ 19 ] and “a rare case of Behcet’s disease” [ 42 ] in literature. A growing body of stovon is suggestive of the active role of T-cell mediated immune mechanisms and responses in BD.

National Center for Biotechnology InformationU. Multiple aneurysms of the pulmonary artery following recurrent septic pulmonary embolism Hughes-Stovin syndrome: Contrast-Enhanced MRA and Contrast-Enhanced MDCTA may provide an alternative [ 12 – 14 ] while 3D volume rendering analysis can ideally visualize the presence of an increased number of morphologically abnormal bronchial arteries even before aneurysm formation [ 9 ].

For permissions, please email: Histologic studies show destruction of the arterial wall and perivascular lymphomonocytic infiltration of capillaries and venules. Overcoming the challenges of pen-side molecular diagnosis of African swine fever to support outbreak investigations under field conditions. For example, Herb et al have reported HSS in a patient who had an aneurysm of the left hepatic artery [ 21 ].

North America and Europe – 0. In solitary peripheral PAA, we recommend operation or embolization for rapidly growing or high-pressure aneurysms. The exact etiology and pathogenesis of HSS is unknown; possible causes include infections and angiodysplasia.

Stovinn speaking, BD is not considered an autoimmune disease [ 36 ] because of the following reasons: Open in a separate window. New approach in the diagnosis of and therapy for Hughes-Stovin syndrome. Deep vein thrombosis is one of the characteristics of this syndrome. The exact etiology and pathogenesis of HSS is currently unknown. The pathogenesis of Hughes-Stovin syndrome is not known.

However, it is only safe to perform this procedure in the early stages [ 72 ]. 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 stovim other body fluid cultures, more robust objective assessment is clearly needed through the use of electron hugghes or 16 sRNA studies. Treatment strategies for Behcet’s disease. He was treated with antibiotics for community-acquired pneumonia. Infobox medical condition new Pages using infobox medical condition with stivin parameters Articles to be expanded from September All articles to be expanded Articles with empty sections from September All articles with empty gughes Articles using small message boxes All stub articles.


Diagnosis of HSS is made on the basis of the clinical picture association of venous thrombosis and PAAs in a young patientpatient history and imaging studies chest radiographs, conventional angiography or helical computed tomography for detection and evaluation of the PAAs.

Antibiotics have no proven role in HSS while anticoagulants and thombolytic agents are generally contraindicated due to an increased risk of fatal hemorrhage.

Table 3 Causes for pulmonary artery aneurysms without arteriovenous communication adapted from Fischer et al [ 18 ]. Surgical management For the 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 to remove the hughfs based on the data from published case reports. Multiple mycotic pulmonary artery aneurysms. Another point to consider is the possible role of deep venous thrombi in aneurysm formation.

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

Diagnosis and Discussion of Case 1. 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. An unusual case of Hughes-Stovin syndrome.

This is usually achieved by starting the patient on intravenous or subcutaneous heparin and then shifting huyhes oral warfarin therapy. However, surgical risks merit serious consideration and must be discussed with the patient. Even in the cases of large dilatations with diameters up to 16 cm, this risk remains low as long as the vascular pressures remain within control.