Abstract
Behçet’s disease (BD) is a systemic inflammatory disease characterized by recurrent oral ulcers, genital ulcers, cutaneous inflammation, and uveitis. In addition, other potentially life-threatening lesions may occur in the intestinal tract, blood vessels, and central nervous system. This heterogeneity of the BD phenotype hampers development of a targeted treatment strategy. The pathogenesis of BD is not fully elucidated, but it is likely that genetically susceptible people develop BD in response to environmental factors, such as microbiome factors. Genetic analyses have identified various BD susceptibility loci that function in HLA-antigen presentation pathways, Th1 and Th17 cells, and autoinflammation related to monocytes/macrophages, or that increase levels of pro-inflammatory cytokines, reduce levels of anti-inflammatory cytokines, or act in dysfunctional mucous barriers. Our functional analyses have revealed that impairment of M2 monocyte/macrophage-mediated anti-inflammatory function through IL-10 is crucial to BD pathogenesis. We, therefore, propose that BD is an M1-dominant disease. In this review, we describe the roles of monocytes and macrophages in BD and consider the potential of these cells as therapeutic targets.
Highlights
Behçet’s disease (BD) is a systemic inflammatory disease initially reported in 1937 by Hulusi Behçet, a prominent Turkish dermatologist
In 2008, we reported the overexpression of TLR4 in peripheral blood mononuclear cells (PBMCs) of BD patients [14]
We addressed how decreased monocyte migration ability revealed by genome-wide association studies (GWAS) is related to the pathogenesis of BD by analyzing the polarization of M1 and M2 macrophages [37]
Summary
Behçet’s disease (BD) is a systemic inflammatory disease initially reported in 1937 by Hulusi Behçet, a prominent Turkish dermatologist. Studies of the involvement of monocytes and macrophages in BD focused on their function as part of the pathogenesis of enhanced neutrophil chemotaxis in response to environmental factors. In a report from 1993, monocytes from patients with active BD displayed increased secretion of pro-inflammatory cytokines, including TNF-a, IL-6, and IL-8 [7].
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
