休斯-斯托文综合征:关于一种罕见的、危及生命的血管炎的病例报告。

Q4 Medicine Mediterranean Journal of Rheumatology Pub Date : 2023-08-29 eCollection Date: 2023-12-01 DOI:10.31138/mjr.290823.hsr
Tayyeba Khursheed, Ahmed Masood, Muhammad Sufyan Khan, Muhammad Sharif, Somaya Shah, Muhammad Arqam Miraj
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引用次数: 0

摘要

休斯-斯托文综合征(HSS)是一种罕见的血管炎性疾病,以广泛的肺动脉瘤为特征。它与贝赫切特病有一些共同特征。目前,诊断主要基于临床怀疑。我们的病例描述的是一名年轻男性,出现咯血并有肺栓塞病史。检查结果基本无异常,但影像学检查发现多发性肺动脉瘤。及时使用糖皮质激素和环磷酰胺进行免疫抑制后,病情有所好转。大剂量糖皮质激素和免疫抑制剂是治疗的主要手段。未经治疗的病例可导致致命后果。
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Hughes-Stovin Syndrome: A Case Report on a Rare, Life-Threatening Vasculitis.

Hughes-Stovin Syndrome (HSS) is a rare vasculitic disorder characterised by widespread pulmonary artery aneurysms. It shares some features with Behçet disease. Currently, the diagnosis is based on clinical suspicion. Our case describes a young male who presented with haemoptysis and previous history of pulmonary embolism. Workup was essentially unremarkable, but imaging revealed multiple pulmonary artery aneurysms. Timely initiation of glucocorticoids and immunosuppression with cyclophosphamide led to improvement. High-dose glucocorticoids and immunosuppressants are the mainstays of treatment. Untreated cases can result in fatal outcomes.

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来源期刊
CiteScore
2.00
自引率
0.00%
发文量
42
审稿时长
8 weeks
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