Behçet's disease – a contemporary review

Abstract

Behçet's disease (BD) is a systemic vasculitis disorder of unknown etiology, characterized by relapsing episodes of oral aphthous ulcers, genital ulcers, skin lesions and ocular lesions. It can affect other systems including vascular, gastrointestinal and neurological systems. It occurs most frequently in an area that coincides with the Old Silk Route (between latitudes 30 degrees and 45 degrees north in Asia and Europe). BD is slightly more frequent and has a worse clinical course in men. It is believed to be due to an auto-immune process triggered by an infectious or environmental agent in a genetically predisposed individual. HLA-B51 is the most strongly associated risk factor. The International Study Group (ISG) for Behçet's Disease created a set of criteria for the diagnosis of BD. Available treatments include corticosteroids, azathioprine, cychlophosphamide, cyclosporine A, interferon-alpha, anti-tumour necrosis factor alpha agents, among others. BD has a variable course characterized by relapses and remissions. Prognosis depends on the clinical involvement. Loss of visual acuity and neurological disease are major causes of morbidity and disability.