Intravenous immunoglobulins (IVIg) represent an established cornerstone for the immunotherapy of chronic inflammatory demyelinating polyneuropathy (CIDP). Efficacy of IVIg for CIDP was proven in a large phase III trial. Yet, data on longer-term efficacy and effects in distinct subgroups are scarce. Our trial investigates the long-term efficacy of IVIg treatment in CIDP patients. In this observational real-world study, we retrospectively analyzed 49 CIDP patients receiving continuous IVIg treatment with a mean initial dosage of 87 g (1 g/kg body weight) every 4 weeks over a mean time of 45 months between 2010 and 2018. INCAT-Scores before the start of treatment and at the end of the observation period were compared. Over the observation period, IVIg treatment led to a median improvement of one INCAT score point. Subgroup analyses revealed a more pronounced improvement of INCAT scores in female CIDP patients, individuals with relapsing disease courses, patients with more pronounced motor impairment (higher initial INCAT scores) and in the cohort without need for concomitant other immunotherapies. These data argue for sustained beneficial effects of longer-term immunotherapy with IVIg in CIDP, particularly in females and relapsing disease forms with higher disease activity.

中文翻译：

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静脉注射免疫球蛋白治疗慢性炎性脱髓鞘性多发性神经病的长期影响：谁受益？

静脉注射免疫球蛋白 (IVIg) 是慢性炎性脱髓鞘性多发性神经病 (CIDP) 免疫治疗的基石。一项大型 III 期试验证明了 IVIg 对 CIDP 的疗效。然而，关于不同亚组的长期疗效和影响的数据很少。我们的试验调查了 IVIg 治疗对 CIDP 患者的长期疗效。在这项观察性的现实世界研究中，我们回顾性分析了 49 名接受连续 IVIg 治疗的 CIDP 患者，平均初始剂量为每 4 周 87 克（1 克/公斤体重），平均时间为 2010 年至 2018 年的 45 个月。 INCAT -比较治疗开始前和观察期结束时的分数。在观察期内，IVIg 治疗导致一个 INCAT 得分点的中值改善。亚组分析显示，女性 CIDP 患者、有复发病程的个体、运动障碍更明显的患者（初始 INCAT 评分较高）和无需伴随其他免疫疗法的队列中的 INCAT 评分改善更显着。这些数据证明了在 CIDP 中使用 IVIg 进行长期免疫治疗的持续有益效果，特别是在女性和疾病活动度较高的复发性疾病中。