Behçet Disease

Abstract

Behçet disease (BD) is a multisystemic autoinflammatory disorder of unknown etiology. BD is most common along the historical Silk Road from Far East Asia to the Mediterranean region. The disease is characterized by recurrent oral/genital ulcers and ocular, gastrointestinal, musculoskeletal, and nervous system involvement. BD is also a unique vasculitis affecting both arteries and veins of all sizes. There are several sets of classification criteria for BD; however, the International Study Group (ISG) diagnostic/classification criteria and the recently proposed pediatric BD (PEDBD) criteria are the most widely used. There are no specific guidelines for the management of pediatric BD; however, very recently, the new European League Against Rheumatism (EULAR) recommendations for adult BD have been proposed.