Autoinflammatory diseases are a group of heritable disorders that are characterized by seemingly unprovoked episodes of inflammation at certain locations and and relative lack of high-titer autoantibodies or antigen-specific T cells. Behçets disease is an inflammatory disorder of unknown aetiology, and many of its characteristic recurrent manifestations overlap with those of autoinflammatory diseases. Behçets disease has a complex genetic aetiology, and it is more prevalent in certain geographic regions and/or in particular ethnic groups. Enhanced inflammatory response and over-expression of proinflammatory cytokines are the prominent features of Behçets disease, and they are compatible with the findings in other autoinflammatory disorders. There are also evidences of antigen-driven immune response in Behçets disease, but it possibly develops on the background of enhanced innate immune reactivity. Delineation of the similarities of Behçets disease to other hereditary autoinflammatory diseases may help to clarify its pathogenesis and also to identify the missing links in the shared inflammatory pathways.
behcets disease, autoinflammatory, familial mediterranean fever, crohn disease, inflammation
Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, 34390 Capa,Istanbul, Turkey.