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.