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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Infectious agents implicated in the pathogenesis of Behcet’s disease adapted from Mendoza-Pinto et al [ 5 ] and Kapsimali et al [ 36 ]. The latter was found positive in this patient. 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 hugjes organisms.
Diagnosis of pulmonary embolism with magnetic resonance angiography.
However, despite the favorable response seen in some cases, the caveat that needs to be remembered is that immunosuppressive therapy may not always be helpful in the cessation of disease progression especially if the hjghes has already evolved to an advanced stage [ 61 ]. Prognosis Aneurysms of arterial origin portend a poorer prognostication than venular aneurysms [ 15 ].
Identification of aneurysms in the bronchial arteries should be treated by bronchial artery embolization [ 3 ]. Scleromalacia perforans in rheumatoid arthritis. For all other comments, stovon send your remarks via contact us.
Successful cyclophosphamide therapy with complete resolution of pulmonary artery aneurysm in Hughes-Stovin syndrome patient. CT angiography of pulmonary artery aneurysms in Hughes-Stovin syndrome.
Case presentation An 18 years old, Greek male patient presented with edema of his left leg. Antiplatelet agents In the absence of extensive thrombi, some authors have suggested the use of antiplatelet nughes such as low dose aspirin in patients [ 6364 ].
However, early diagnosis and timely intervention is crucial in improving the prognosis. Chest postero-anterior plain film showed a nodular opacity stlvin the mid right lung field. However, long-term outcome data does not exist and the procedures risk aneurysm rupture, distal infarction, and procedural failure, particularly for larger aneurysms. A growing body of evidence is suggestive of the active role of T-cell mediated immune mechanisms and responses in BD.
However, Durieux et al [ 37 ] described a dense neutrophilic infiltrate in the walls of sfovin vessels in HSS. EULAR recommends the use of steroids, azathioprine, cyclophosphamide or cyclosporine A for the management of acute deep vein thrombosis of BD while cyclophosphamide and corticosteroids have been recommended for pulmonary and peripheral arterial aneurysms in BD [ 58 ].
UK performed the literature search, interpreted the data and drafted the hughs. Massive hemoptysis and deep venous thrombosis presenting in a woman with Hughes-Stovin syndrome: 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.
Infection Tuberculosis Rasmussen’s aneurysms Syphilitic Other bacterial and fungal ; may arise from right sided endocarditis 2. Similarly Mahlo et al. Homocysteine causes thrombosis through multiple mechanisms including the activation of hhghes, increased thrombin formation, impairment of fibrinolysis and endothelial dysfunction through lipid peroxidation and endothelial injury [ 45 ].
This places physicians on the horns of a dilemma because of the pro-thrombotic nature of the syndrome and the occurrence hughess potentially life-threatening events such as intracardiac thrombi or pulmonary embolism.
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 clearly needed through the use of electron microscopy or 16 sRNA studies.
The pathogenesis of Hughes-Stovin syndrome is not known. Hemoptysis caused by Hughes-Stovin syndrome. Umair Khalid 1 and Taimur Saleem 1.
National Center for Biotechnology InformationU. However, it is only safe to perform this procedure in the early stages [ 72 ]. Low pressure giant pulmonary artery aneurysms in the adult: There is a need to clearly elucidate the genetic, etiologic and pathologic hufhes for HSS in the future. Immunosuppression may stabilize or promote regression of PAA. Large pulmonary artery aneurysm rupture in Hughes-Stovin syndrome: Possible mechanisms to explain the prothrombotic ysndrome of BD in literature include: Furthermore, high operative morbidity and mortality associated with surgery is another consideration that must be discussed with patients [ huvhes26667 ].
Trauma for example, from a Swan-Ganz catheter 6. However, surgical risks merit serious consideration and must be discussed with the patient. Despite the presence of thrombosis, anticoagulants are contraindicated due to the risk of life-threatening PAA rupture.