Update on the Therapy of Behçet's Disease

Behçet's disease is a chronic inflammatory systemic disorder, characterized by a relapsing and remitting course. The main histopathological finding is a widespread vasculitis of the arteries and veins of any size. The prevalence of BD is highest in the Middle East, Mediterranean region and Asia. The usual age of onset is around 30, and the male to female ratio varies with the ethnic background. The cause of BD remains unknown. It is presumed to be multifactorial involving infectious triggers, genetic predisposition, and dysregulation of the immune system. The main objectives of therapy of BD are to relieve its symptoms, decrease recurrences and prevent irreversible damage. This choice, however, is limited by the paucity of high quality therapy trials. Therapy studies for this disease in general, lack high methodological quality and common outcome measures, making treatment of this disease for the most part empirical. Most randomized studies have focused on the treatment of ocular, mucocutaneous and musculoskeletal diseases, while data on the treatment of the other manifestations is based on uncontrolled data.