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Abstract：
Behcet\'s disease (BD) is a rare but globally distributed vasculitis that primarily affects populations in the Mediterranean and Asian regions. Behcet\'s uveitis (BU) is a common manifestation of BD, occurring in over two-thirds of the patients. BU is characterized by bilateral, chronic, recurrent, non-granulomatous uveitis in association with complications such as retinal ischemia and atrophy, optic atrophy, macular ischemia, macular edema, and further neovascular complications (vitreous hemorrhage, neovascular glaucoma). Although the etiology and pathogenesis of BU remain unclear, numerous studies reveal that genetic factors (such as HLA-B51), dysregulated immune responses of both the innate and adaptive immune systems, infections (such as streptococcus), and environmental factors (such as GDP) are all involved in its development. Innate immunity, including hyperactivity of neutrophils and γδT cells and elevated NK1/NK2 ratios, has been shown to play an essential role in this disease. Adaptive immune system disturbance, including homeostatic perturbations, Th1, Th17 overaction, and Treg cell dysfunction, is thought to be involved in BU pathogenesis. Treatment of BU requires a tailored approach based on the location, severity of inflammation, and systemic manifestations. The therapy aims to achieve rapid inflammation suppression, preservation of vision, and prevention of recurrence. Systemic corticosteroids combined with other immunosuppressive agents have been widely used to treat BU, and beneficial effects are observed in most patients. Recently, biologics have been shown to be effective in treating refractory BU cases. Novel therapeutic targets for treating BU include the LCK gene, Th17/Treg balance, JAK pathway inhibition, and cytokines such as IL-17 and RORγt. This article summarizes the recent studies on BU, especially in terms of pathogenesis, diagnostic criteria and classification, auxiliary examination, and treatment options. A better understanding of the significance of microbiome composition, genetic basis, and persistent immune mechanisms, as well as advancements in identifying new biomarkers and implementing objective quantitative detection of BU, may greatly contribute to improving the adequate management of BU patients.
摘要：
Behcet病（BD）是一种罕见但全球分布的血管炎，主要影响地中海和亚洲地区的人群。白塞氏葡萄膜炎(BU)是BD的常见表现,发生在超过三分之二的患者中。BU的特点是双边，慢性，经常性,与视网膜缺血和萎缩等并发症相关的非肉芽肿性葡萄膜炎，视神经萎缩,黄斑缺血,黄斑水肿,和进一步的新生血管并发症（玻璃体出血，新生血管性青光眼）。尽管BU的病因和发病机制尚不清楚,大量研究表明，遗传因素(如HLA-B51)，先天和适应性免疫系统的免疫反应失调，感染（如链球菌），和环境因素(如GDP)都参与了它的发展。先天免疫力,包括中性粒细胞和γδT细胞的过度活跃和NK1/NK2比率升高，已被证明在这种疾病中起着至关重要的作用。适应性免疫系统紊乱，包括稳态扰动，Th1，Th17过度反应，和Treg细胞功能障碍，被认为与BU发病机制有关。BU的治疗需要一种基于位置的量身定制的方法，炎症的严重程度，和系统性表现。该疗法旨在实现快速的炎症抑制，保护视力,预防复发。全身性皮质类固醇联合其他免疫抑制剂已被广泛用于治疗BU，在大多数患者中观察到有益效果。最近,生物制剂已被证明可有效治疗难治性BU病例。用于治疗BU的新治疗靶标包括LCK基因,Th17/Treg平衡，JAK通路抑制,和细胞因子如IL-17和RORγt。本文总结了BU的最新研究，特别是在发病机制方面，诊断标准和分类，辅助检查,和治疗选择。更好地理解微生物组组成的重要性，遗传基础,和持续的免疫机制，以及在识别新的生物标志物和实施BU客观定量检测方面的进步，可能大大有助于改善BU患者的适当管理。
