Heat Shock Proteins in Behçet Syndrome

Behçet syndrome (BS) is a systemic vasculitis of unknown etiology that affects the skin, mucosa, joints, eyes, central nervous system, gastrointestinal system, arteries, and veins. It is generally believed to have a complex genetic background where both innate and adaptive immune systems are activated through environmental factors, such as infections, and auto-antigens. Heat shock proteins (HSPs) are highly conserved and immunogenic endogenous proteins that are thought to play both an enhancing and regulating role in several autoimmune and inflammatory diseases, such as rheumatoid arthritis, juvenile idiopathic arthritis, and Type I diabetes. There is evidence supporting the role of various microorganisms in BS, which may be using a common pathway to trigger or activate BS through molecular mimicry. The significant homology between microbial and human HSPs suggests that HSPs could serve as a common trigger. This review summarizes the work on the role of HSPs in the pathogenesis of BS. However, it remains unknown whether the HSPs detected in BS lesions play a causative role, their presence is a result of the ongoing inflammation, or they have a protective role against inflammation, as suggested in some other diseases.