[Pathophysiology of Behçet's disease].

Abstract

Although the precise pathogenesis and etiology of Behçet's disease (BD) still remains unknown, current evidence suggests that inflammatory reaction in BD arises from disruption of homeostasis in genetically susceptible individuals, resulting in altered innate and adaptive immunity responses, pathogenic T cell activation in the peripheral blood, and in inflammatory sites. Association with HLA-B51 is known as the strongest genetic susceptibility factor for BD. Recent GWAS (genome-wide association studies) have confirmed this relationship, and reported new susceptibility genes (IL-10, IL-23R, IL-12RB2) for the disease. A triggering infectious agent could operate through molecular mimicry, and the disease could subsequently be perpetuated by an abnormal immune response to an auto-antigen in the absence of ongoing infection. Several potential bacteria have been investigated but the most commonly implicated microorganism is Streptococcus sanguis. Recent data have showed that the T cell homeostasis perturbation consisted mainly of Th1 and Th17 expansions, while regulatory T cell response was suppressed. Cytokine such as IL-17, IL-23 and IL-21 play a significant role in the pathogenesis of BD. Inflammatory cells within BD inflammatory lesions include mostly neutrophils, CD4(+) T cells, and cytotoxic cells. Lastly, endothelium dysfunction has been clearly established. This improved understanding of the pathophysiology of BD will certainly lead to the development of new therapeutic agents, potentially more effective than current therapy. In this review, we have studied the etiopathogenesis of BD in the light of recent advances.

• Authors: M H Houman, N Bel Feki
• Journal: La Revue de medecine interne (Rev Med Interne) Vol. 35 Issue 2 Pg. 90-6 (Feb 2014) ISSN: 1768-3122 [Electronic] France
• Vernacular Title: Physiopathologie de la maladie de Behçet.
• PMID: 24210264 (Publication Type: English Abstract, Journal Article, Review)
• Copyright: Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

Topics

• Behcet Syndrome (etiology, genetics, immunology)
• Communicable Diseases (complications, immunology)
• Environment
• Genetic Predisposition to Disease
• Humans
• Immune System (physiology)