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Review
. 2023 Aug:253:109661.
doi: 10.1016/j.clim.2023.109661. Epub 2023 Jun 7.

Immunopathogenesis of Behçet's disease

Arshed F Al-Obeidi  1 , Johannes Nowatzky  2
Affiliations
Review

Immunopathogenesis of Behçet's disease

Arshed F Al-Obeidi et al. Clin Immunol. 2023 Aug.

Abstract

Behçet's disease (BD) is a multi-system inflammatory disorder with vasculitic features. It does not suit any of the current pathogenesis-driven disease classifications well, a unifying concept of its pathogenesis is not unanimously conceivable at present, and its etiology is obscure. Still, evidence from immunogenetic and other studies supports the notion of a complex-polygenic disease with robust innate effector responses, reconstitution of regulatory T cells upon successful treatment, and first clues to the role of an, as of yet, underexplored adaptive immune system and its antigen recognition receptors. Without an attempt to be comprehensive, this review aims to collect and organize impactful parts of this evidence in a way that allows the reader to appreciate the work done and define the efforts needed now. The focus is on literature and notions that drove the field into new directions, whether recent or more remote.

Keywords: Antigen-recognition; Behcet; CD8 T cells; ERAP1; HLA-B51; NK cells.

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Conflict of interest statement

Declaration of Competing Interest None.

Figures

Figure 1:
Figure 1:
Most polymorphisms linked to BD risk are shared with other diseases, especially Recurrent Aphthous Stomatitis (bold genes at the bottom of the pyramid), or can be mimics of BD (e.g., SJS involving the mucosal membranes; HLA-B*27-associated diseases; IBD, PS). However, epistasis of HLA-B*51 with ERAP1-Hap10 recessively confers the highest BD risk known to date and is BD ‘specific.’ The haplotype ERAP1-Hap10, associated with BD risk in the presence of HLA-B*51, protects from AS/AAU and PS in the presence of their HLA class I risk alleles, i.e., HLA-B*24 and HLA-C*06, respectively. (AS = ankylosing spondylitis; AAU = acute anterior uveitis; IBD = inflammatory bowel diseases; PBC = primary biliary cirrhosis; PS = psoriasis; SJS = Stevens-Johnson syndrome) Figure created with BioRender.com
Figure 2:
Figure 2:. Timeline of selected mile stones in BD discovery.
Refers to results from [, , , , , , , –84] Figure created with BioRender.com
See this image and copyright information in PMC

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