We herein to describe the response and the potential treatment mechanism of low dose rituximab in patients with treatment-resistant chronic inflammatory demyelinating polyneuropathy (CIDP) with antibodies against neurofascin-155 (NF-155). Patients received 100 mg rituximab once weekly for 4 weeks followed by 100 mg per month for 2 additional doses. Clinical function scores, Fahn- Tolosa-Marin Tremor Rating Scale (FTMTRS) and flow cytometry of peripheral blood were scheduled before and at 1, 3, 6 months after rituximab treatment. All clinical function score including MRC, INCAT, Hughes, mRS, ODSS and FTMTRS scores showed obvious improvement at the post-treatment follow-up 1,3,6 months in comparison with baseline values. The proportion of CD19 + CD27+, CD19 + CD38+ and CD138 in lymphocytes of all patients declined at 1,3,6 month and the proportion of CD19 + CD24hiCD38hi in one patient was increased at 6 months after rituximab treatment. Low dose rituximab can significant improve disease severity and disabling tremor of CIDP patients with anti-NF155 antibody by the powerful role of B cell depletion within six months and subsequent reestablishment of B-cell subsets including increasing regulatory B cells, inhibiting memory B cells and reducing plasmablasts.

中文翻译：

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低剂量利妥昔单抗在具有抗 NF-155 抗体的难治性 CIDP 中的疗效

我们在此描述了低剂量利妥昔单抗对具有抗神经纤维蛋白 155 (NF-155) 抗体的难治性慢性炎性脱髓鞘性多发性神经病 (CIDP) 患者的反应和潜在治疗机制。患者每周接受一次 100 毫克利妥昔单抗，持续 4 周，然后每月接受 100 毫克额外剂量。临床功能评分、Fahn-Tolosa-Marin 震颤评定量表（FTMTRS）和外周血流式细胞术分别在利妥昔单抗治疗前和治疗后 1、3、6 个月进行。与基线值相比，所有临床功能评分包括 MRC、INCAT、Hughes、mRS、ODSS 和 FTMTRS 评分在治疗后随访 1、3、6 个月均显示出明显改善。所有患者淋巴细胞中CD19+CD27+、CD19+CD38+和CD138的比例下降至1,3，6个月，1例患者CD19+CD24hiCD38hi的比例在利妥昔单抗治疗后6个月增加。低剂量利妥昔单抗可通过六个月内 B 细胞耗竭和随后重建 B 细胞亚群（包括增加调节性 B 细胞、抑制记忆 B 细胞和减少）的强大作用，显着改善具有抗 NF155 抗体的 CIDP 患者的疾病严重程度和致残性震颤。浆母细胞。