Pathophysiology of the Behçet's Disease

Abstract

Behçet's disease (BD) is a multisystemic disease of unknown etiology characterized by chronic relapsing oral-genital ulcers and uveitis. Multiple systemic associations including articular, gastrointestinal, cardiopulmonary, neurologic, and vascular involvement are also observed in BD. Although the etiopathogenesis of the disease remains unknown, increased neutrophil functions such as chemotaxis, phagocytosis, and excessive production of reactive oxygen species (ROS), including superoxide anion, which may be responsible for oxidative tissue damage seen in BD, and also immunological alterations, T lymphocyte abnormalities in both subpopulation and function have been considered to be correlated with the etiopathogenesis of BD. There is some clinical evidence suggesting that emotional stress and hormonal alterations can influence the course and disease activity of BD.

Figure 1

Proposed model of the pathogenesis in Behçet's disease. Ag: antigen; APC: antigen-presenting cells; HSP: heat shock protein; IFN: interferon; IL: interleukin; IPP: isoprenyl pyrophosphate; PPP: prenyl pyrophosphate; TCR: T-cell receptor; Th1: T-helper cells type 1; TNF-α: tumor necrosis factor α.