Faculty of Medicine, Department of Neurology, Uludag University, Bursa, Turkey.
Bursa Şevket Yılmaz Training and Research Hospital, Neurology, Bursa, Turkey.
Clin Rheumatol. 2024 Oct;43(10):3213-3221. doi: 10.1007/s10067-024-07118-9. Epub 2024 Aug 25.
Behcet's syndrome is a rare inflammatory disorder characterized by oral and genital ulcers, skin lesions, and uveitis. It exhibits a higher prevalence along the historic Silk Road. Neuro-Behcet syndrome (NBS) affects the central nervous system and poses significant morbidity and mortality risks. Infliximab, a TNF-alpha antagonist, has shown potential in NBS management, although the current evidence is mainly derived from case series due to the lack of randomized controlled trials.
This retrospective study aimed to evaluate the disease outcomes during the first and second years following infliximab treatment in NBS patients experiencing attacks despite prior conventional immunosuppressive therapy. The study also sought to investigate the safety profile and adverse effects associated with infliximab.
Fifty-three NBS patients were examined, with 22 receiving infliximab as either monotherapy or in combination with other therapies. Retrospective analysis was conducted on demographic data, clinical characteristics, and treatment responses. Treatment efficacy was measured using the Expanded Disability Status Scale (EDSS) modified for NBS. The study adhered to the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) checklist guidelines.
Among the study cohort, 60.4% had parenchymal NBS, and 39.6% had nonparenchymal NBS. Treatment with infliximab resulted in remission or disease stabilization in 95% of patients after one year and 68.7% after 2 years. Relapse rates were 4.5% at 1 year and 18.7% at 2 years, with disease progression observed in two cases. Adverse effects were primarily mild to moderate, with no reports of serious adverse events.
Infliximab exhibited efficacy in achieving remission or stabilization in NBS patients, maintaining a favorable safety profile. The timing of infliximab treatment may prevent the accumulation of disability and hinder disease progression. Nonetheless, future prospective studies are necessary to confirm these findings and refine treatment strategies for this complex condition.
贝赫切特综合征是一种罕见的炎症性疾病，其特征为口腔和生殖器溃疡、皮肤损伤和葡萄膜炎。它在历史丝绸之路沿线的发病率更高。神经贝赫切特综合征（NBS）影响中枢神经系统，存在较高的发病率和死亡率风险。英夫利昔单抗是一种 TNF-α拮抗剂，已显示出在 NBS 管理中的潜力，尽管由于缺乏随机对照试验，目前的证据主要来自病例系列研究。
本回顾性研究旨在评估在经历发作的 NBS 患者中，尽管先前接受了常规免疫抑制治疗，但在使用英夫利昔单抗治疗的第一和第二年的疾病结局。该研究还旨在调查英夫利昔单抗相关的安全性概况和不良反应。
检查了 53 名 NBS 患者，其中 22 名患者接受英夫利昔单抗单药或联合其他治疗。对人口统计学数据、临床特征和治疗反应进行回顾性分析。使用改良的用于 NBS 的扩展残疾状况量表（EDSS）来衡量治疗效果。该研究遵循观察性研究的报告强化标准（STROBE）清单指南。
在研究队列中，60.4%的患者为实质 NBS，39.6%的患者为非实质 NBS。英夫利昔单抗治疗一年后，95%的患者和两年后 68.7%的患者病情缓解或稳定。一年时的复发率为 4.5%，两年时为 18.7%，有两例出现疾病进展。不良反应主要为轻度至中度，无严重不良事件报告。
英夫利昔单抗在 NBS 患者中能实现缓解或稳定，具有良好的安全性。英夫利昔单抗治疗的时机可能会阻止残疾的累积并阻止疾病进展。然而，仍需要未来的前瞻性研究来证实这些发现并完善对这种复杂疾病的治疗策略。
