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休斯-斯托文综合征:一例罕见的、危及生命的血管炎病例报告。

Hughes-Stovin Syndrome: A Case Report on a Rare, Life-Threatening Vasculitis.

作者信息

Khursheed Tayyeba, Masood Ahmed, Khan Muhammad Sufyan, Sharif Muhammad, Shah Somaya, Miraj Muhammad Arqam

机构信息

Department of Rheumatology, Pakistan Institute of Medical Sciences, Islamabad, Pakistan.

出版信息

Mediterr J Rheumatol. 2023 Aug 29;34(4):565-569. doi: 10.31138/mjr.290823.hsr. eCollection 2023 Dec.

Abstract

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.

摘要

休斯-斯托文综合征(HSS)是一种罕见的血管炎疾病,其特征为广泛的肺动脉瘤。它与白塞病有一些共同特征。目前,诊断基于临床怀疑。我们的病例描述了一名年轻男性,他出现咯血且有既往肺栓塞病史。检查基本无异常,但影像学显示有多个肺动脉瘤。及时开始使用糖皮质激素和环磷酰胺进行免疫抑制治疗后病情有所改善。大剂量糖皮质激素和免疫抑制剂是主要治疗方法。未经治疗的病例可能导致致命后果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6498/10815522/279886d2885c/MJR-34-4-565-g001.jpg

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