Behçet's disease: successful treatment with infliximab in 7 patients with severe vascular manifestations. A retrospective analysis.

OBJECTIVE: To evaluate the therapeutic effect of infliximab in patients with inflammatory vascular lesions due to Behçet's disease (BD).

METHODS: Seven patients with clinical evidence of severe vascular BD were analyzed: 3 patients with aortic involvement, 1 with recurrent venous thrombosis of the pelvic veins, 1 with recurrent venous and arterial thromboses of the thigh, and 2 with retinal vasculitis. Infliximab was initiated with 3-5 mg/kg of body weight and infusions were repeated in intervals of 4 weeks as either first-line therapy in 3 patients or add on after failure of conventional immunosuppression in the remaining 4. Ongoing immunosuppression consists of a various combination of azathioprine (n = 2), methotrexate (n = 3), cyclosporine (n = 3), and low-dose glucocorticoids (n = 3).

RESULTS: Control of inflammation was seen 1-5 days after infliximab induction in all patients. C-reactive protein level was reduced from a mean of 89 mg/liter prior to infliximab to 9 mg/liter thereafter. Vision increased rapidly in patients with retinal vasculitis. Vascular grafts remained patent. The inflamed and dissected aortic wall healed over a period of 6 months. Infliximab could be stopped in 2 patients; intervals could be extended in 4 to a maximum of 8 weeks. Infliximab and basic immunosuppression were well tolerated; no drug-induced side effects were recorded.

CONCLUSION: Infliximab is effective in inducing and maintaining remission of vasculitic activity in patients with BD. The rapid effect together with excellent tolerability suggests that infliximab should be considered as a first-line agent in severe vascular BD.