Risk factors, clinical features and treatment of Behçet's disease uveitis

IF 18.6 1区 医学 Q1 OPHTHALMOLOGY
Zhenyu Zhong, Guannan Su, Peizeng Yang
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引用次数: 0

Abstract

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.

Behçet病葡萄膜炎的危险因素、临床特点及治疗。
Behçet病是一种经常与眼内炎症相关的系统性血管炎。最近的研究结果确定了Behçet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Behçet病主要表现为葡萄膜炎,其特征是一个独立的集群,与其他系统相关的可能性很低,如肠道、心血管或中枢神经系统。一种流行的理论认为,这种疾病的发病机制是多因素的,各种遗传和传染因素可能相互作用导致疾病。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,已被发现是遗传易感性的关键组成部分。在HLA区域之外,发现了一组与白细胞介素(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的易感性变体。微生物感染,如血链球菌、结核分枝杆菌和单纯疱疹病毒(HSV),与免疫遗传学易感个体的疾病触发有关。临床上,由于眼部Behçet病有明显的复发-缓解过程,预防复发发作将是主要治疗目标。皮质类固醇和免疫调节药物的组合,如抗TNF药物、干扰素和常规免疫抑制剂(如环孢菌素、硫唑嘌呤),已成为该疾病的主流治疗方案。未来的研究可能侧重于比较免疫调节药物的有效性,并在了解疾病分子异质性的基础上确定最适合特定药物的亚组。
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来源期刊
CiteScore
34.10
自引率
5.10%
发文量
78
期刊介绍: Progress in Retinal and Eye Research is a Reviews-only journal. By invitation, leading experts write on basic and clinical aspects of the eye in a style appealing to molecular biologists, neuroscientists and physiologists, as well as to vision researchers and ophthalmologists. The journal covers all aspects of eye research, including topics pertaining to the retina and pigment epithelial layer, cornea, tears, lacrimal glands, aqueous humour, iris, ciliary body, trabeculum, lens, vitreous humour and diseases such as dry-eye, inflammation, keratoconus, corneal dystrophy, glaucoma and cataract.
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