BEHCETS-SYNDROME - HOW SHOULD WE TREAT IT

Behcet's syndrome is a multisystem vasculitis of unknown aetiology. Eye involvement, the main cause of morbidity, can lead to blindness in 20% of those affected. Other lesions, ranging from aphthous and genital ulceration to sometimes fatal central nervous system involvement, also cause considerable morbidity and, we have become more recently aware, mortality. The syndrome runs a course of exacerbations and remissions, and usually abates in intensity with the passage of time. Young adult males have the worst prognosis. The main aim of treatment is to prevent irreversible organ damage during th early, active, phase of the disease. Immunosuppression remains the mainstay of therapy. Azathioprine is able to suppress most of the manifestations of the syndrome. Cyclosporin has a considerably more rapid onset of action, and is particularly useful in the treatment of uveitis. However, the disease usually flares on cessation of cyclosporin treatment. Neither azathioprine nor cyclosporin is always effective, and there are patients who continus to do badly even with their combined use. Thalidomide is useful in severe oral ulceration and colchicine in erythema nodosum associated with Behcet's syndrome. There is no established remedy for the central nervous system and thrombotic complications of Behcet's syndrome.