Behçet's disease: Current therapeutic perspectives

Behçet's disease is a chronic relapsing systemic vasculitis of small and large vessels, of unknown etiology, characterized by recurrent oral and genital ulcers, and ocular inflammation. Other involved organs may include the joints, skin, and gastrointestinal tract, as well as the pulmonary, renal, central nervous, and the cardiovascular systems. The treatment of Behçet's disease is basically empiric, geared to control the symptoms, suppress the inflammatory process, and prevent organ damage. Thus, various antiinflammatory and immunosuppressive agents have been used as the main therapeutic modalities, although none of them has been effective in controlling the diverse symptoms. Earlier, these drugs included colchicine, azathioprine, systemic corticosteroids, dapsone, cyclosporine A, and tacrolimus. Recently emerging studies show the effectiveness of thalidomide, interferon alpha, interferon gamma, acyclovir, low-dose weekly methotrexate, cyclophosphamide pulse therapy, and anti-tumor necrosis factor (TNF) therapy. Anti-TNF agents, such as infliximab, are emerging as being effective in rapid control of sight-threatening panuveitis and cerebral vasculitis, as well as in orogenital ulcerations, gastrointestinal disease, and skin lesions of Behçet's disease. Limited anecdotal case reports have been published with the use of the fusion protein etanercept in Behçet's disease. Hopefully, prospective clinical trials, which are now ongoing, will provide more data as to the efficacy of the anti-TNF agents, compared with the conventional immunosuppressive therapy.