Departments of Neurology (B.Z., U.U., S.S., O.N.D., A.S.) and Rheumatology (E.S., S.U., V.H.), Istanbul University, Cerrahpasa School of Medicine, Istanbul, Turkey; and Department of Neurology (B.Z., O.H.K.), Mayo Clinic College of Medicine, Rochester, MN.
Neurol Neuroimmunol Neuroinflamm. 2016 Jul 8;3(5):e258. doi: 10.1212/NXI.0000000000000258. eCollection 2016 Oct.
We evaluated the effectiveness of infliximab in patients with neuro-Behçet syndrome for whom other immunosuppressive medications had failed.
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.
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.
We observed a significant beneficial effect of infliximab in neuro-Behçet syndrome.
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.
评估英夫利昔单抗在其他免疫抑制剂治疗失败的复发性神经白塞病患者中的疗效。
对于复发性神经白塞病且其他免疫抑制剂治疗失败的患者，需要选择替代药物。我们报告了肿瘤坏死因子α抑制剂英夫利昔单抗用于长期治疗神经白塞病的经验。我们在白塞病多学科转诊中心招募患者，并前瞻性随访所有出现神经系统症状的患者。在接受另一种免疫抑制剂治疗时出现≥2 次神经系统发作（不包括单纯进行性疾病）的患者（n=16）改用英夫利昔单抗治疗，并成功维持治疗（0、2 和 6 周时给予 5mg/kg，之后每 8 周 1 次；最低随访时间≥12 个月）。在 1 例患者中，英夫利昔单抗治疗开始后 2 个月因肺和中枢神经系统结核而停药。
在改用英夫利昔单抗之前，患者因神经白塞病扩展残疾状况量表评分逐渐加重而出现疾病复发（评分中位数为 5.0，范围为 2.0-7.0；神经白塞病病程中位数为 29.1 个月，范围为 5.0-180.7）。在使用英夫利昔单抗之前，所有患者免疫抑制剂的中位使用时间为 20.0 个月（范围为 3.0-180.7）。在所有 15 例患者中，在英夫利昔单抗治疗期间（评分中位数为 4.0，范围为 2.0-7.0；病程中位数为 39.0 个月，范围为 16.0-104.9 个月），神经系统复发完全停止，且无进一步残疾加重。
我们观察到英夫利昔单抗在神经白塞病中具有显著的有益作用。
本研究提供了 IV 级证据，表明对于其他免疫抑制剂治疗失败的神经白塞病患者，英夫利昔单抗可预防进一步复发并稳定残疾。
