Behçet's disease as an autoinflammatory disorder

Abstract

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. Behçet's disease is an inflammatory disorder of unknown aetiology, and many of its characteristic recurrent manifestations overlap with those of autoinflammatory diseases. Behçet's 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 Behçet's disease, and they are compatible with the findings in other autoinflammatory disorders. There are also evidences of antigen-driven immune response in Behçet's disease, but it possibly develops on the background of enhanced innate immune reactivity. Delineation of the similarities of Behçet's disease to other hereditary autoinflammatory diseases may help to clarify its pathogenesis and also to identify the missing links in the shared inflammatory pathways.