Management of Behcet's syndrome

Abstract

Behcet's syndrome (BS) is a variable vessel vasculitis with heterogeneous clinical features. Skin, mucosa and joint involvement can cause impairment of quality of life but do not cause permanent damage whereas untreated eye, vascular, nervous system and gastrointestinal system involvement can cause serious damage and even death. Management of BS as a multidisciplinary team enables a faster and more accurate diagnosis and well-integrated treatment strategies. Corticosteroids are the mainstay of therapy. Colchicine, AZA, ciclosporin-A, cyclophosphamide, IFN alpha, and tumour necrosis factor alpha inhibitors are other agents used as induction and/or maintenance therapy. Although biologic agents have been increasingly used, there are still unmet needs. Head-to-head comparison studies of some therapeutic options (e.g. TNF inhibitors vs IFN alpha in uveitis) are required. Novel therapeutic agents in the pipeline could change the standard of care for BS in the future.

Keywords: Behcet’s disease; Behcet’s syndrome; Neurobehcet’s syndrome; anti-TNF agents; biologic agents; interferon alpha; uveitis; vascular Behcet’s syndrome.