PMID- 37734442 OWN - NLM STAT- MEDLINE DCOM- 20231110 LR - 20231114 IS - 1873-1635 (Electronic) IS - 1350-9462 (Linking) VI - 97 DP - 2023 Nov TI - Risk factors, clinical features and treatment of Behcet's disease uveitis. PG - 101216 LID - S1350-9462(23)00055-1 [pii] LID - 10.1016/j.preteyeres.2023.101216 [doi] AB - Behcet's disease is a systemic vasculitis frequently associated with intraocular inflammation. Recent findings identified independent clinical clusters in Behcet's disease, each involving distinct combinations of affected organs. Ocular Behcet'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 Behcet'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. CI - Copyright (c) 2023 Elsevier Ltd. All rights reserved. FAU - Zhong, Zhenyu AU - Zhong Z AD - The First Affiliated Hospital of Chongqing Medical University, Chongqing Key Laboratory of Ophthalmology, Chongqing Eye Institute, Chongqing Branch (Municipality Division) of National Clinical Research Center for Ocular Diseases, Chongqing, China. FAU - Su, Guannan AU - Su G AD - The First Affiliated Hospital of Chongqing Medical University, Chongqing Key Laboratory of Ophthalmology, Chongqing Eye Institute, Chongqing Branch (Municipality Division) of National Clinical Research Center for Ocular Diseases, Chongqing, China. FAU - Yang, Peizeng AU - Yang P AD - The First Affiliated Hospital of Chongqing Medical University, Chongqing Key Laboratory of Ophthalmology, Chongqing Eye Institute, Chongqing Branch (Municipality Division) of National Clinical Research Center for Ocular Diseases, Chongqing, China. Electronic address: peizengycmu@126.com. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20230919 PL - England TA - Prog Retin Eye Res JT - Progress in retinal and eye research JID - 9431859 RN - 0 (Immunomodulating Agents) RN - 0 (Tumor Necrosis Factor Inhibitors) SB - IM MH - Humans MH - *Behcet Syndrome/drug therapy/genetics MH - Immunomodulating Agents MH - Tumor Necrosis Factor Inhibitors/therapeutic use MH - *Uveitis/drug therapy/etiology MH - Risk Factors OTO - NOTNLM OT - Behcet's disease OT - Clinical management OT - Diagnosis OT - Ocular disease OT - Risk factors OT - Uveitis COIS- Declaration of competing interest The authors declare no competing financial interests. EDAT- 2023/09/22 00:42 MHDA- 2023/11/10 06:44 CRDT- 2023/09/21 19:13 PHST- 2023/06/21 00:00 [received] PHST- 2023/09/09 00:00 [revised] PHST- 2023/09/15 00:00 [accepted] PHST- 2023/11/10 06:44 [medline] PHST- 2023/09/22 00:42 [pubmed] PHST- 2023/09/21 19:13 [entrez] AID - S1350-9462(23)00055-1 [pii] AID - 10.1016/j.preteyeres.2023.101216 [doi] PST - ppublish SO - Prog Retin Eye Res. 2023 Nov;97:101216. doi: 10.1016/j.preteyeres.2023.101216. Epub 2023 Sep 19.