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白塞综合征

Behçet syndrome.

作者信息

Espinosa Gerard

机构信息

Servicio de Enfermedades Autoinmunes, Centro de Referencia (UEC/CSUR) en Enfermedades Autoinmunes Sistémicas, Vasculitis y Enfermedades Autoinflamatorias; Miembro de ERN-ReCONNET/RITA; Hospital Clínic, Barcelona, España; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, España; Facultat de Medicina i Ciències de la Salut; Universitat de Barcelona, Barcelona, España.

出版信息

Med Clin (Barc). 2025 Jul;165(1):106983. doi: 10.1016/j.medcli.2025.106983. Epub 2025 May 15.

DOI:10.1016/j.medcli.2025.106983
PMID:40378634
Abstract

Behçet's syndrome (BS) is a systemic vasculitis that affects vessels of all calibers. It has several defining characteristics, such as its tendency for remission over time and a typical geographical distribution. Clinically, the association of venous thrombosis with arterial aneurysms, inflammatory parenchymal brain involvement, the classic pattern of posterior uveitis with retinal vasculitis, and the well-known triad of bipolar ulcers and erythema nodosum are distinctive features of this condition. Despite some advances in the pathogenesis of BS and the use of biological drugs that have improved prognosis, certain aspects remain controversial, such as the role of the pathergy test or the determination of HLA-B*51 in the diagnosis of the disease, or the actual value of anticoagulation in patients with BS and vascular thrombosis.

摘要

白塞病(BS)是一种影响各种管径血管的系统性血管炎。它有几个典型特征,比如有随时间缓解的倾向以及典型的地理分布。临床上,静脉血栓形成与动脉动脉瘤相关、炎症性脑实质受累、伴有视网膜血管炎的典型后葡萄膜炎模式,以及众所周知的口腔溃疡、结节性红斑三联征是这种疾病的显著特征。尽管在白塞病发病机制方面取得了一些进展,并且使用生物药物改善了预后,但某些方面仍存在争议,比如同形反应试验的作用、HLA - B*51在疾病诊断中的测定,或者白塞病合并血管血栓形成患者抗凝治疗的实际价值。

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