The pathogenic mechanisms in Behçet's syndrome are largely unknown. An autoantigen role for human leukocyte antigen B51 has been proposed. The reasons behind the thrombophilia are also not clear. Endothelial pathology could be the main culprit. The recently proposed association between familial Mediterranean fever and Behçet's syndrome might not be well founded. The long-term prognosis is more guarded among the young and among males. However, the disease burns out in many cases. Clinicians are getting better at management, and have better understanding of the old drugs, such as colchicine, and have new and potent drugs like tumor necrosis factor-alpha inhibitors.