Update on the epidemiology, risk factors and disease outcomes of Behçet's disease.

Behçet's disease (BD) may be regarded as a polygenic autoinflammatory disease although adaptive immune system has also been implicated in pathogenesis. Different classification criteria sets exist for BD, including the new "International Criteria for BD." The pooled prevalence of BD was calculated as 10.3 per 100,000 population globally. BD is common along the Silk Road, including Turkey. Male sex and early onset are associated with a more severe disease course. For the follow-up of BD, there are five disease activity scales, one disease severity scale, and one QOL scale.