Behçet's disease: New insights into pathophysiology, clinical features and treatment options

Publication date: June 2018Source: Autoimmunity Reviews, Volume 17, Issue 6Author(s): Antonio Greco, Armando De Virgilio, Massimo Ralli, Andrea Ciofalo, Patrizia Mancini, Giuseppe Attanasio, Marco de Vincentiis, Alessandro LambiaseAbstractBehçet's disease (BD) is a rare systemic vasculitis characterized by oral aphthous ulcers, genital ulcers, ocular lesions and other systemic manifestations. BD occurs most frequently in Eurasian populations along the ancient trading route known as the “Silk Road” which extends from eastern Asia to the Mediterranean basin. The causes of BD are unknown: it is believed to be due to an autoimmune process triggered by an infectious or environmental agent in genetically predisposed individuals. HLA-B51 allele located in the MHC locus, on chromosome 6p, has been the most strongly associated risk factor for BD in areas along the Old Silk Route. Herpes simplex virus-1 and Streptococcus have been postulated as possible environmental triggers of BD. T cell homeostasis perturbation, especially Th1 and Th17 expansion and decreased regulation by Tregs are now supposed to be the cornerstone of BD pathogenesis. The histology shows vasculitis that involves both arteries and veins, and vessels of any size. BD is a systemic vasculitis with significant neutrophil infiltration, endothelial cell swelling, and fibrinoid necrosis. The diagnosis of BD is only supported by clinical criteria and requires the exclusion of other diagnoses based on clinical presenta...
Source: Autoimmunity Reviews - Category: Allergy & Immunology Source Type: research