Epidemiology of Behçet Syndrome

Behçet disease (BD) is a systemic inflammatory disease typically characterized by oral and genital ulcers and variable manifestations affecting other organs, mainly skin and eye. Published epidemiologic studies on BD are difficult to compare because of different study designs, settings, methods and the lack of universally accepted classification criteria: 16 different sets of classification/diagnosis criteria have been published so far; in the beginning of the chapter we compare different criteria subsets with estimates of sensitivity and specificity. After that, we review epidemiologic clinical studies (registries, hospital records, district databases): consistent data point to higher incidence and prevalence rates of BD along the ancient Silk Road (stretching from the Middle Est to the Far East and Mediterranean Countries) compared to a lower prevalence in Northern European Countries and United States genetic and environmental factors have been evaluated as well as age of onset, male-to-female ratio and systems involvement.