Review paper Insights into Behçet’s disease

Abstract

Behcet’s disease is a chronic relapsing multi-organ inflammatory disorder characterized as a triad of oral and gen ital ulcers, uveitis. Characteristic manifestations of Behcet’s disease are joints, skin, central nervous system and gas trointestinal tract involvement. Behcet’s disease has a complicated genetic etiology. However, epidemiological stud ies recommend that genetic factors have a significant role in its pathogenesis, the same as other autoinflammatory disorders. Antigenic stimuli, antigen-presenting cells, T cells, monocyte, neutrophil and endothelial cells are most important parts of the pathology of the disease. Inflammatory response was triggered by an infectious agent in a genetically susceptible host. Understanding the pathogenesis based on the molecular mechanism of the disease highlights the new therapeutic modalities. Enhanced inflammatory activity and over-expression of proinflammato ry cytokines are the striking features of Behcet’s disease, and they are accordant with the result in other auto inflam matory disorders. Moreover, there is evidence of antigen-driven immune response in Behcet’s disease, but it prob ably advances in further innate immune reactivity. New therapeutic modalities target specific and nonspecific suppression of the immune system. The diagnosis is a clinical one, and although there is no single laboratory test enough for the diagnosis of Behcet’s disease. In this paper, a new aspect of the studies on genetic susceptibility, immunopathogenesis of Behcet’s disease and novel treatment modalities will be discussed.