Behçet's Disease (Adamantiades-Behçet's Disease)

Abstract

Adamantiades-Behçet's disease (ABD) is characterized by starting with oral aphthous ulceration and developing of the systemic involvements. The pathogenesis of ABD is closely correlated with the genetic factors and the triggering factors which acquire delayed-type hypersensitivity reaction against oral streptococci mediated by IL-12 cytokine family. HLA-B51 is associated in more than 60% of the patients and its restricted CD8+ T cell response is clearly correlated with the target tissues. Bes-1 gene encoded partial S. sanguinis genome which is highly homologous with retinal protein, and 65 kD heat shock protein (Hsp-65) released from streptococci is playing an important role with human Hsp-60 in the pathogenesis of ABD. Although Hsp-65/60 has homologies with the respective T cell epitope, it stimulates peripheral blood mononuclear cells (PBMCs) from ABD patients. On the other hand, some peptides of Hsp-65 were found to reduce IL-8 and IL-12 production from PBMCs of ABD patients in active stage.

Common MEFV gene mutations in Turkish patients with Behcet's disease

“…Etiopathogenesis of BD has not been fully elucidated, but genetic and environmental factors might play critical role (Borlu et al, 2006;Gül, 2005). None of the specific genetic factors, such as HLA-B51, TLR gene, or IL-12B, is fully associated with BD (Hornung et al, 2002;Kaneko et al, 2011;Yanagihori et al, 2006).…”

MDR1 gene polymorphisms may be associated with Behçet's disease and its colchicum treatment response

“…Previous studies have reported the presence of an association between BD and genetic factors. Among these, HLA-B51 has been reported to be significantly related to this disease state (Kaneko et al, 2011;Ohno et al, 1982). In addition, increased TLR gene expression (Hornung et al, 2002) and IL-12B gene polymorphism (Yanagihori et al, 2006) were indicated to be associated with BD.…”

Behcet’s Disease and Related Diseases -Immune Reactions to Oralstreptococci in Their Pathogenesis

“…The APCs impair MICA expressed endothelium of the vessels in correlation with HSP-65/60, VEGF, adhesion molecules, etc. BD lesions will be induced by the "vascular reaction" and/or "lymphocytic vasculitis" as the immunological reactions due to the APCs expressing S. sanguinis antigen [97,98] ( Figure 8). …”