Treatment of Behcet's disease - An update

Objectives: To report the experience of the investigators and review the ma jor treatment trials conducted for Behcet's disease (BD). Methods: A MEDLINE literature review from 1970 to date was performed on the drugs prescribed for the treatment of BD. Open and controlled clinical stu dies and indications for the treatment of affected organs are analyzed. Results: Glucocorticoids are indicated for the treatment of BD, although no controlled studies have been reported. The combination of corticosteroids and immunosuppressant drugs is used when vital organs are involved. Nonster oidal anti-inflammatory drugs are of little value in arthritis. In controll ed trials, colchicine was efficacious for erythema nodosum and arthritis, p articularly in women. Cyclosporine A has a rapid action and when combined w ith azathioprine is effective in patients with severe uveitis and extraocul ar manifestations. Chlorambucil is indicated for uveitis and meningoencepha litis. In controlled studies, azathioprine prevented unilateral uveitis fro m becoming bilateral and improved extraocular symptoms. Pulse cyclophospham ide combined with corticosteroids improves severe systemic vasculitis. Inte rferon alpha benefits ocular and extraocular manifestations, but controlled studies are lacking. Methotrexate is indicated for uveitis and arthritis, and sulfasalazine improves gastrointestinal vasculitis. In controlled trial s, thalidomide was effective for mucocutaneous manifestations, but on its d iscontinuation the disease exacerbated. Orogenital manifestations are treat ed with local application of corticosteroids or other medications. Conclusions: Combination therapy is not always efficacious in controlling i nflammation. The goal of management is to treat early to avoid recurrences and irreversible damage to the vital organs. With proper management of BD, loss of useful vision was reduced from 75% to 20% of the affected eyes. How ever, less favorable results are seen for central nervous system and large artery and vein involvement. Semin Arthritis Rheum 30:299-312. Copyright (C) 2001 by W.B. Saunders Compa ny.