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休斯-斯托文综合征:肺动脉瘤和血栓形成的罕见病因——病例报告

Hughes-Stovin Syndrome: A Rare Cause of Pulmonary Artery Aneurysm and Thrombosis-A Case Report.

作者信息

Qadir Abdul, Abdellatif Amal Wael, Waheed Aamir, Islam Mamunul

机构信息

Department of Medicine, Hamad Medical Corporation HGH, Doha, Qatar.

Medical Education Department, Hamad Medical Corporation, Doha, Qatar.

出版信息

Int J Rheum Dis. 2025 Apr;28(4):e70227. doi: 10.1111/1756-185X.70227.

Abstract

Hughes-Stovin Syndrome (HSS) is a rare, life-threatening condition characterized by thrombophlebitis and pulmonary artery aneurysms, often viewed as a variant of Behçet's disease. Its diagnosis and management are challenging due to its rarity and overlapping symptoms with other vascular disorders. A 33-year-old male presented with massive hemoptysis and a history of recurrent oral and scrotal ulcers. Imaging revealed bilateral pulmonary emboli, a pulmonary artery aneurysm, and a large right atrial thrombus. These findings, coupled with his clinical history, led to a diagnosis of HSS. A multidisciplinary team initiated anticoagulation for the thrombus and immunosuppressive therapy (steroids, cyclophosphamide). Follow-up imaging showed improvement in both the aneurysm and thrombus. This case highlights the complexity of diagnosing and managing HSS. Early recognition, supported by a collaborative approach, is critical to improving outcomes in this rare syndrome.

摘要

休斯-斯托文综合征(HSS)是一种罕见的、危及生命的疾病,其特征为血栓性静脉炎和肺动脉瘤,常被视为白塞病的一种变体。由于其罕见性以及与其他血管疾病症状重叠,其诊断和治疗颇具挑战性。一名33岁男性出现大量咯血,并有复发性口腔和阴囊溃疡病史。影像学检查显示双侧肺栓塞、一个肺动脉瘤和一个巨大的右心房血栓。这些发现,结合他的临床病史,导致诊断为HSS。一个多学科团队开始对血栓进行抗凝治疗,并给予免疫抑制治疗(类固醇、环磷酰胺)。随访影像学检查显示动脉瘤和血栓均有改善。该病例突出了HSS诊断和治疗的复杂性。在协作方法的支持下尽早识别,对于改善这种罕见综合征的治疗效果至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3355/11995141/bb0d3c994af4/APL-28-e70227-g002.jpg

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