Department of Allergy and Rheumatology, Hospital for Skin Diseases, Institute of Dermatology, Chinese Academy of Medical Sciences, Peking Union Medical College, 12 Jiangwangmiao Street, Nanjing, 210042, China.
Clin Rev Allergy Immunol. 2021 Dec;61(3):363-376. doi: 10.1007/s12016-021-08864-3. Epub 2021 Jun 2.
Behcet's syndrome (BS) is a chronic systemic inflammatory vasculitis with a wide range of clinical manifestations including recurrent oral and genital ulcers; cutaneous lesions; and ophthalmic, neurologic, and gastrointestinal involvement. BS has a global distribution but is particularly prevalent in so-called Silk Road populations. Disease onset is usually around the third or fourth decade of life, and the sex ratio is roughly 1:1. Both environmental and genetic factors contribute to the etiology of BS, although the detailed mechanisms remain unclear. At present, there is no laboratory examination with diagnostic value for BS; therefore, a diagnosis is made based on clinical manifestations. The International Study Group diagnostic criteria published in 1990 is the most widely used and recognized, but in order to improve sensitivity, the International Criteria for Behcet's Disease is developed in 2014. Evaluating disease activity in BS is an important basis for treatment selection and monitoring, the simplified Behcet's Disease Current Activity Form (2006 version) is a well-established scoring method. Given that multiple organs are affected in BS, it must be differentiated from other diseases with similar manifestations or that may be induced by drug treatment. The goal of BS treatment is to eradicate triggers and/or aggravating factors, alleviate and control clinical symptoms, prevent and treat any damage to organs, slow disease progression, and improve the patient's quality of life. The clinical management of BS depends on the affected organs and disease severity. In this review, we summarize the current state of knowledge of BS pathogenesis and therapeutic options.
白塞病（BS）是一种慢性全身性炎症性血管炎，具有广泛的临床表现，包括反复发作的口腔和生殖器溃疡；皮肤损害；以及眼部、神经系统和胃肠道受累。BS 具有全球分布，但在所谓的丝绸之路人群中更为流行。发病通常在生命的第三或第四个十年左右，性别比例大致为 1:1。环境和遗传因素都与 BS 的病因有关，尽管详细机制尚不清楚。目前，BS 没有具有诊断价值的实验室检查；因此，根据临床表现做出诊断。1990 年发表的国际研究组诊断标准是应用最广泛和公认的，但为了提高敏感性，2014 年开发了国际白塞病诊断标准。评估 BS 的疾病活动是治疗选择和监测的重要基础，简化的白塞病当前活动评分表（2006 版）是一种成熟的评分方法。鉴于 BS 多器官受累，必须与具有相似表现或可能由药物治疗引起的其他疾病相鉴别。BS 治疗的目标是消除诱因和/或加重因素，缓解和控制临床症状，预防和治疗任何器官损害，减缓疾病进展，提高患者的生活质量。BS 的临床管理取决于受累器官和疾病严重程度。在这篇综述中，我们总结了 BS 发病机制和治疗选择的最新知识。
