Behçet's disease is a systemic vasculitis frequently associated with intraocular inflammation. Recent findings identified independent clinical clusters in Behçet's disease, each involving distinct combinations of affected organs. Ocular Behçet's disease, mainly manifested as uveitis, is characterized as an independent cluster with a low likelihood of association with other system involvements, such as intestinal, cardiovascular, or central nervous system. A prevailing theory suggests that the pathogenesis of the disease is multifactorial, where a variety of genetic and infectious agents may interact with each other to cause the disease. Among sporadic cases, the human leukocyte antigen (HLA) genes, including HLA-B51, HLA-A26, HLA-B15, and HLA-B5701, have been found to be a key component conferring genetic susceptibility. Outside the HLA region, a set of susceptibility variants are identified, closely related to interleukin (IL)-23/IL-17 pathway, tumor necrosis factor (TNF) signaling, and pattern recognition receptor systems. Microbial infections, such as Streptococcus sanguinis, Mycobacterium tuberculosis, and Herpes simplex virus (HSV), are linked to play the triggering of disease in immunogenetically predisposed individuals. Clinically, due to the notable relapsing-remitting course of ocular Behçet's disease, the prevention of recurrent attack would be the primary treatment goal. Combination of corticosteroids and immunomodulatory drugs, such as anti-TNF agents, interferon, and conventional immunosuppressants (e.g. cyclosporine, azathioprine), have been the mainstream regimen for the disease. Future research may focus on comparing the effectiveness of immunomodulatory drugs and identifying the most suitable subgroups for a specific drug on the basis of the knowledge of the molecular heterogeneity of the disease.

白塞氏病是一种系统性血管炎，通常与眼内炎症相关。最近的研究结果确定了白塞氏病的独立临床集群，每个集群都涉及受影响器官的不同组合。眼部白塞氏病主要表现为葡萄膜炎，其特征是独立的集群，与其他系统受累（如肠道、心血管或中枢神经系统）相关的可能性较低。一种流行的理论认为，该疾病的发病机制是多因素的，多种遗传因素和感染因素可能相互作用而导致该疾病。在散发病例中，人类白细胞抗原（HLA）基因，包括HLA-B51、HLA-A26、HLA-B15和HLA-B5701，被发现是赋予遗传易感性的关键成分。在 HLA 区域之外，鉴定了一组易感性变异，它们与白细胞介素 (IL)-23/IL-17 通路、肿瘤坏死因子(TNF) 信号传导和模式识别受体系统密切相关。微生物感染，如血链球菌、结核分枝杆菌和单纯疱疹病毒 (HSV)，与免疫遗传易感个体的疾病触发有关。临床上，由于眼白塞病具有显着的复发缓解病程，预防复发是首要治疗目标。皮质类固醇与免疫调节药物（如抗TNF药物、干扰素）和常规免疫抑制剂（如环孢素、硫唑嘌呤）的联合治疗已成为该病的主流治疗方案。未来的研究可能集中于比较免疫调节药物的有效性，并根据疾病分子异质性的知识确定特定药物最合适的亚组。