Behçet's syndrome is a disease of unknown aetiology classified among the
vasculitides. It runs a course of exacerbations and remissions which gradually abate with time.
Eye disease, the most frequent cause of serious morbidity, may lead to
blindness in 20% of those affected. The syndrome may occasionally be fatal due to
vasculitis leading to
arterial occlusion, ruptured arterial
aneurysms or pulmonary
vasculitis, or involvement of the central nervous system. Immunosuppressive drugs have been shown to be moderately successful in inducing and maintaining remissions.
Azathioprine at a dose of 2.5 mg/kg/day has been shown to control the progression of existing, and the development of new,
eye disease.
Cyclosporin A is also beneficial in controlling active
eye disease and although it has a more rapid action than
azathioprine, its toxicity limits its long term use.
Colchicine, although widely prescribed, has been shown in a controlled trial to be effective only in reducing the development of
erythema nodosum and
arthralgia. Systemic
corticosteroids, once widely used, are now reserved only for the most severe cases of inflammatory
eye disease and
vasculitis, where they are frequently used as intravenous pulse
therapy. Local
mydriatics are used to prevent synechiae. Local treatment with
corticosteroids, sometimes in conjunction with
antibiotics, control oral and genital
ulcers which may also be controlled by immunosuppressives, which are reserved for the most severe cases.
Thrombophlebitis usually only requires
antiplatelet agents, whereas
arteritis is treated conventionally with a combination of
corticosteroids and immunosuppressive drugs, usually
cyclophosphamide.