Treatment of behçet’s disease - an update

Objectives: To report the experience of the investigators and review the major treatment trials conducted for Behçet'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 studies 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. Nonsteroidal anti-inflammatory drugs are of little value in arthritis. In controlled trials colchicine was efficacious for erythema nodosum and arthritis particularly in women. Cyclosporine A has a rapid action and when combined with azathioprine is effective in patients with severe uveitis and extraocular manifestations. Chlorambucil is indicated for uveitis and meningoencephalitis. In controlled studies azathioprine prevented unilateral uveitis from becoming bilateral and improved extraocular symptoms. Pulse cyclophosphamide combined with corticosteroids improves severe systemic vasculitis. Interferon α benefits ocular and extraocular manifestations but controlled studies are lacking. Methotrexate is indicated for uveitis and arthritis and sulfasalazine improves gastrointestinal vasculitis. In controlled trials thalidomide was effective for mucocutaneous manifestations but on its discontinuation the disease exacerbated. Orogenital manifestations are treated with local application of corticosteroids or other medications. Conclusions: Combination therapy is not always efficacious in controlling inflammation. 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. However less favorable results are seen for central nervous system and large artery and vein involvement.