Etiopathogenesis of Behçet’s syndrome: The role of infectious, genetic, and immunological environmental factors

This chapter introduces current knowledge regarding the etiopathogenesis of Behçet’s syndrome (BS). It focuses on the classification of BS, highlighting how it shares common features with autoimmune diseases, autoinflammatory diseases, and MHC-I-opathies. The chapter subsequently presents the main individual and exogenous factors involved in the etiopathogenesis of BS. Particularly, it explains the contribution of microbial triggers, genetic susceptibility, epigenetics, and immunological factors to the etiopathogenesis of BS. Understanding the etiopathogenesis of BS is essential to identify modifiable risk factors for the occurrence or exacerbation of the disease and to develop targeted treatments.