Abstract
OBJECTIVE: We evaluated the effectiveness of infliximab in patients with neuro-Behçet syndrome for whom other immunosuppressive medications had failed. METHODS: Patients whose common immunosuppressive medications fail in recurrent neuro-Behçet syndrome need an alternative. We report our experience with the tumor necrosis factor α blocker infliximab for long-term treatment of neuro-Behçet syndrome. We recruited patients within a multidisciplinary referral practice of Behçet disease and prospectively followed everyone with a neurologic symptom(s). Patients (n = 16) with ≥2 neurologic bouts (excluding purely progressive disease) while on another immunosuppressive treatment were switched to and successfully sustained on infliximab (5 mg/kg in weeks 0, 2, and 6, then once every 8 weeks; minimum follow-up duration ≥12 months). Infliximab was stopped within 2 months after initiation in one patient because of pulmonary and CNS tuberculosis. RESULTS: Patients had stepwise worsening due to relapses in the Expanded Disability Status Scale modified for neuro-Behçet syndrome before switching to infliximab (median score of 5.0, range 2.0-7.0; median neuro-Behçet syndrome duration 29.1 months, range 5.0-180.7). Median duration of preinfliximab immunosuppressive medication use was 20.0 months (range 3.0-180.7). In all 15 patients, during infliximab treatment (median score 4.0, range 2.0-7.0; median duration 39.0 months, range 16.0-104.9 months), neurologic relapses were completely aborted and there was no further disability accumulation. CONCLUSION: We observed a significant beneficial effect of infliximab in neuro-Behçet syndrome. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that for patients with neuro-Behçet syndrome whose other immunosuppressive medications failed, infliximab prevents further relapses and stabilizes disability.