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Retrospective analysis of response to rituximab in chronic inflammatory demyelinating polyneuropathy refractory to first-line therapy
Journal of the Peripheral Nervous System ( IF 3.8 ) Pub Date : 2021-09-01 , DOI: 10.1111/jns.12461
Farzad Fatehi 1 , Ali Asghar Okhovat 1 , Akram Panahi 1 , Bentolhoda Ziaaddini 1 , Yusuf A Rajabally 1, 2 , Shahriar Nafissi 1
Affiliation  

Few case reports/series describe the efficacy of rituximab in refractory chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which is preferred in the presence of anti-nodal/paranodal antibodies. We aimed at evaluating the clinical response to rituximab in a subset of patients with refractory CIDP for whom the anti-nodal/paranodal antibodies status was unknown, as not available in Iran. We retrospectively analyzed the response to rituximab in 14 Iranian patients with refractory CIDP (3 children, 11 adults), in whom the anti-nodal/paranodal antibodies status was unknown. The subjects were evaluated with the Medical Research Council (MRC) sum score (MRCSS), Inflammatory Neuropathy Cause and Treatment (INCAT) disability scores, and electrophysiology, before and after treatment. Mean age was 34.4 ± 20.7 years, disease duration pre-rituximab treatment was 27.8 ± 18.8 (range: 6-60) months, and mean follow-up duration was 18.5 ± 11.0 (range: 4-36) months. Considering the INCAT sum score, one worsened during post-rituximab treatment, and three patients did not change. Considering MRCSS, notably, four patients achieved normalization of their MRCSS. Regarding the corticosteroid dose, two patients could discontinue prednisolone. As rated by a pre-defined scoring system, nerve conduction parameters improved significantly post-rituximab in the treated cohort (P = .006). All patients tolerated rituximab infusions without adverse effects. Rituximab may be effective in refractory CIDP, even though worsening may occur in some patients. Anti-nodal/paranodal antibodies assay, when available, and other criteria may help drive therapeutic decision-making on rituximab as second-line treatment.

中文翻译:

一线治疗无效的慢性炎症性脱髓鞘性多发性神经病对利妥昔单抗反应的回顾性分析

很少有病例报告/系列描述了利妥昔单抗在难治性慢性炎症性脱髓鞘性多发性神经根神经病 (CIDP) 中的疗效,在存在抗结节/结节旁抗体的情况下,这是首选。我们的目的是评估抗结节/结节旁抗体状态未知的难治性 CIDP 患者亚组对利妥昔单抗的临床反应,因为在伊朗不可用。我们回顾性分析了 14 名伊朗难治性 CIDP 患者(3 名儿童,11 名成人)对利妥昔单抗的反应,这些患者的抗结节/结节旁抗体状态未知。受试者在治疗前后使用医学研究委员会 (MRC) 总分 (MRCSS)、炎症性神经病病因和治疗 (INCAT) 残疾评分以及电生理学进行评估。平均年龄为 34.4 ± 20.7 岁,利妥昔单抗治疗前的病程为27.8±18.8(范围:6-60)个月,平均随访时间为18.5±11.0(范围:4-36)个月。考虑到 INCAT 总分,1 名患者在利妥昔单抗治疗期间恶化,3 名患者没有变化。值得注意的是,考虑到 MRCSS,4 名患者的 MRCSS 实现了正常化。关于皮质类固醇剂量,两名患者可以停用泼尼松龙。根据预先定义的评分系统评估,治疗组中的神经传导参数在接受利妥昔单抗治疗后显着改善(4 名患者的 MRCSS 恢复正常。关于皮质类固醇剂量,两名患者可以停用泼尼松龙。根据预先定义的评分系统评估,治疗组中的神经传导参数在接受利妥昔单抗治疗后显着改善(4 名患者的 MRCSS 恢复正常。关于皮质类固醇剂量,两名患者可以停用泼尼松龙。根据预先定义的评分系统评估,治疗组中的神经传导参数在接受利妥昔单抗治疗后显着改善(P  = .006)。所有患者均耐受利妥昔单抗输注,无不良反应。利妥昔单抗可能对难治性 CIDP 有效,尽管某些患者可能会出现恶化。抗结节/结节旁抗体测定(如果有)和其他标准可能有助于推动利妥昔单抗作为二线治疗的治疗决策。
更新日期:2021-09-01
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