A modern view of the etiology, epidemiology and clinical manifestations of Behcet’s disease

Abstract

Behcet’s disease (BD) is a systemic vasculitis of unknown etiology, which is characterized by of variable vessel involvement, and the main clinical manifestations are recurrent oral aphthous ulcers and genital sores, ocular, joints, gastrointestinal tract and central nervous system lesions. Although BD is increasingly being diagnosed and effectively treated, the etiology of this disease remains unclear. In addition, there is a difficulty in developing universal classification criteria, which is associated with geographical differences in the prevalence, severity of certain clinical signs and the disease severity. This literature review provides the latest data on the epidemiology, etiopathogenesis, and clinical manifestations of BD. Both certain or «strong» signs for the diagnosis of BD, as well as probable or «weak» are presented. «Strong» criteria are features that define the BD as a separate nosological unit, that is, a pathological condition with a unique pathogenesis and more or less unique features that distinguish it from other pathological conditions. On the contrary, «weak» symptoms are those elements that indicate more than one common pathogenetic mechanism with another disease. Diagnosis of BD is even more complicated by the absence of specific histopathological or laboratory components that can be used in diagnosis. Further research and clinical work are needed with an emphasis on understanding phenotypic expression and the geographical impact on BD development.