英夫利西单抗治疗重症和(或)难治性神经白塞病的疗效及安全性

Infliximab for the treatment of severe and/or refractory neuro-Behcet’ disease

目的探讨英夫利西单抗(infliximab,IFX)治疗重症和(或)难治性神经白塞病(neuro-Behcet’disease,NBD)的疗效和安全性。方法回顾性分析2016年1月至2019年12月在北京协和医院接受IFX治疗的10例重症和(或)难治性NBD患者的临床资料。结果 10例患者均为实质受累NBD,8例为多发病灶,6例同时并发脑干、大脑半球受累,起病时Rankin评分(4.1±0.88),且伴发活动性眼炎和肠道溃疡各1例。应用IFX联合激素和免疫抑制剂治疗中位疗程6(1, 40)月,9例患者治疗有效,神经系统症状改善,Rankin评分降至(2.7±1.34)分(P=0.000 5),且4例(40%)患者影像学病变较前减轻。IFX治疗后炎症指标较治疗前显著下降,中位血沉由16(10, 70)mm/h下降至5(1, 13)mm/h(P=0.007),中位C反应蛋白由7.53(1.86, 60)mg/L降至1.35(0.51, 5.0)mg/L(P=0.005)。此外,患者口腔、外阴溃疡、皮肤病变、关节炎、葡萄膜炎、肠道溃疡均明显改善。中位随访时间13(4, 40)月,1例完全缓解,8例部分缓解,糖皮质激素使用剂量较前显著减少,泼尼松由(60.0±21.73)mg降至(21.25±14.78)mg(P=0.000 2)。结论 IFX联合传统治疗可有效治疗重症和(或)难治性NBD。

Objective To investigate the effectiveness and safety of Infliximab(IFX) in the treatment for severe and/or refractory neuro-Behcet’ disease(NBD). Methods The clinical data of ten patients with severe and/or refractory NBD who received IFX treatment in Peking Union Medical College Hospital from January 2016 to December 2019 were retrospectively analyzed. Results All patients had parenchymal NBD, eight patients had multiple lesions, and six had concurrent brainstem and hemicerebrum involvement. The Rankin score at the onset of disease was(4.1±0.88). Active uveitis and intestinal ulcer were reported in one patient each. The median course of treatment with IFX combined with glucocorticoids and immunosuppressants was 6(1, 40)months. Effectiveness was observed in nine patients with evidence included neurological symptoms relieving, Rankin score(2.7±1.34) points decreasing(P= 0.000 5), and imaging lesions improving in four patients(40 %). After IFX treatment, the inflammatory biomarkers decreased significantly as compared with that of baseline. The median ESR decreased from 16(10, 70)mm/h to 5(1, 13) mm/h(P=0.007), and median C-reactive protein decreased from 7.53(1.86, 60)mg/L to 1.35(0.51, 5.0)mg/L(P=0.005). In addition, oral and genital ulcers, skin lesions, arthritis, uveitis, and intestinal ulcers improved accordingly. During a median follow-up time of 13(4, 40)months, one patient achieved complete remission and, eight patients had a partial response. The dose of glucocorticoids tapered from(60.0±21.73)mg to(21.25±14.78)mg(prednisone equivalent)(P=0.000 2). Conclusion In combination with conventional therapy, IFX was effective in the treatment of severe and/or refractory NBD.