Abstract
Behçet’s disease (BD) is a chronic refractory multisystem autoinflammatory disease, characterized by typical clinical features of non-specific vasculitis, oral and genital ulcers, uveitis, as well as skin lesions. The exact etiopathogenesis of BD remains unknown, existing studies have indicated that genetics and environmental factors contribute to the increased development of BD. Recently, several studies have shown that external environmental factors can affect the process of epigenetic modification, and abnormalities of epigenetic factors have been confirmed to be involved in the occurrence of BD. At the same time, abnormalities of gut microbiota (GM) in the body, have also been confirmed to participate in the pathogenesis of BD by regulating the balance of Th17/Tregs. This article reviews the pathogenesis of BD and summarizes numerous clinical studies, focusing on the mechanism of GM and epigenetic factors impacting on BD, and providing new ideas for further elucidating the pathogenesis of BD.
Highlights
Behçet’s disease (BD) is a recurrent, chronic, multisystem autoinflammatory disease, characterized by recurrent stomatitis, uveitis, genital ulcer, oral ulcer, and skin damages (Alipour et al, 2017; Ortiz-Fernández and Sawalha, 2021)
Accumulating evidence has shown that many genetic factors, such as HLA-B51, IL1A-IL1B, CEBPB-PTPN1, IRF8, ADOEGR2, RIPK2, and LACC1, are involved in the susceptibility of BD, the exact etiology of BD remains unclear (Takeuchi et al, 2017)
SUMO4 participates in autoimmunity and inflammation by regulating NF-κB and activating heat shock transcription factors, resulting in the decreased transcription of proinflammatory cytokines (Hou et al, 2008), some studies have shown that SUMO4 +438 C and −847 G alleles seem to be associated with susceptibility to BD, and their gene polymorphisms may be involved in the development of skin lesions, vascular BD, as well as the severity of the disease (Kamoun et al, 2010)
Summary
Behçet’s disease (BD) is a recurrent, chronic, multisystem autoinflammatory disease, characterized by recurrent stomatitis, uveitis, genital ulcer, oral ulcer, and skin damages (Alipour et al, 2017; Ortiz-Fernández and Sawalha, 2021). Barnesiellaceae may exert protective anti-inflammatory effects by reducing the level of TNF-α, one of the key and targeted cytokines of BD, and the decrease in butyric acid production may be regulated by reducing the abundance of Lachnospira, thereby affecting T-cell differentiation and causing inflammation in BD.
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