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白塞综合征。

Behçet Syndrome.

机构信息

Division of Rheumatology, Department of Internal Medicine, Istanbul University - Cerrahpasa, School of Medicine, Istanbul, Turkey; Behçet's Disease Research Center, Istanbul University - Cerrahpasa, Istanbul, Turkey.

Behçet's Disease Research Center, Istanbul University - Cerrahpasa, Istanbul, Turkey; Department of Ophthalmology, Istanbul University - Cerrahpasa, School of Medicine, Istanbul, Turkey.

出版信息

Rheum Dis Clin North Am. 2023 Aug;49(3):585-602. doi: 10.1016/j.rdc.2023.03.010. Epub 2023 May 2.

Abstract

Behçet's syndrome is a systemic vasculitis affecting arteries and veins of all sizes as well as recurrent oral, genital, and intestinal ulcers, skin lesions, predominantly posterior uveitis, and parenchymal brain lesions. These can be present in various combinations and sequences over time and diagnosis is made by recognizing the manifestations, as there are no diagnostic biomarkers or genetic tests. Treatment modalities include immunomodulatory agents, immunosuppressives and biologics, tailored according to prognostic factors, disease activity, severity, and patients' preferences.

摘要

白塞综合征是一种系统性血管炎,影响所有大小的动脉和静脉,以及反复发作的口腔、生殖器和肠道溃疡、皮肤损伤,主要是后葡萄膜炎和实质脑病变。这些可以在不同的时间以不同的组合和顺序出现,通过识别表现来做出诊断,因为目前没有诊断生物标志物或基因检测。治疗方法包括免疫调节剂、免疫抑制剂和生物制剂,根据预后因素、疾病活动度、严重程度和患者偏好进行定制。

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