Data_Sheet_1_Insights into refractory chronic inflammatory demyelinating polyneuropathy: a comprehensive real-world study.PDF

BackgroundRefractory chronic inflammatory demyelinating polyneuropathy (CIDP) is a challenging subset of CIDP. It does not respond well to immune therapy and causes substantial disability. A comprehensive understanding of its clinical profile, electrophysiological characteristics and potential risk factors associated with refractoriness remains to be further elucidated. MethodsData in this cross-sectional study was collected and reviewed from the Huashan Peripheral Neuropathy Database (HSPN). Included patients were categorized into refractory CIDP and non-refractory CIDP groups based on treatment response. The clinical and electrophysiological characteristics were compared between refractory and non-refractory CIDP groups. Potential risk factors associated with refractory CIDP were explored with a multivariate logistic regression model. ResultsFifty-eight patients with CIDP were included. Four disease course patterns of refractory CIDP are described: a relapsing–remitting form, a stable form, a secondary progressive form and a primary progressive form. Compared to non-refractory CIDP patients, refractory CIDP exhibited a longer disease duration (48.96 ± 33.72 vs. 28.33 ± 13.72 months, p = 0.038) and worse functional impairment (MRC sum score, 46.08 ± 12.69 vs. 52.81 ± 7.34, p = 0.018; mRS, 2.76 ± 0.93 vs. 2.33 ± 0.99, p = 0.082; INCAT, 3.68 ± 1.76 vs. 3.03 ± 2.28, p = 0.056, respectively). Electrophysiological studies further revealed greater axonal impairment (4.15 ± 2.0 vs. 5.94 ± 2.77 mv, p = 0.011, ulnar CMAP) and more severe demyelination (5.56 ± 2.86 vs. 4.18 ± 3.71 ms, p = 0.008, ulnar distal latency, 7.94 ± 5.62 vs. 6.52 ± 6.64 ms, p = 0.035, median distal latency; 30.21 ± 12.59 vs. 37.48 ± 12.44 m/s, p = 0.035, median conduction velocity; 58.66 ± 25.73 vs. 42.30 ± 13.77 ms, p = 0.033, median F-wave latency), compared to non-refractory CIDP. Disease duration was shown to be an independent risk factor for refractory CIDP (p < 0.05, 95%CI [0.007, 0.076]). ConclusionThis study provided a comprehensive description of refractory CIDP, addressing its clinical features, classification of clinical course, electrophysiological characteristics, and prognostic factors, effectively elucidating its various aspects. These findings contribute to a better understanding of this challenging subset of CIDP and might be informative for management and treatment strategies.【背景】难治性慢性炎症性脱髓鞘性多发性神经病（refractory chronic inflammatory demyelinating polyneuropathy, CIDP）是慢性炎症性脱髓鞘性多发性神经病（chronic inflammatory demyelinating polyneuropathy, CIDP）中极具挑战性的亚型。该亚型对免疫治疗应答不佳，可导致严重的功能残疾。目前学界对其临床特征、电生理特点以及与难治性相关的潜在危险因素的全面认识仍有待进一步阐明。 【方法】本横断面研究的数据采集自华山周围神经病数据库（Huashan Peripheral Neuropathy Database, HSPN）并进行回顾性分析。根据治疗应答情况，入组患者被分为难治性CIDP组与非难治性CIDP组。对比两组患者的临床特征与电生理特点，并通过多因素logistic回归模型探讨与难治性CIDP相关的潜在危险因素。 【结果】本研究共纳入58例CIDP患者，并描述了难治性CIDP的4种临床病程类型：复发缓解型、稳定型、继发性进展型与原发性进展型。与非难治性CIDP患者相比，难治性CIDP患者的病程更长（48.96±33.72 对 28.33±13.72 个月，p=0.038），功能缺损更显著：MRC总和评分分别为46.08±12.69与52.81±7.34（p=0.018）；改良Rankin量表（mRS）评分分别为2.76±0.93与2.33±0.99（p=0.082）；INCAT评分分别为3.68±1.76与3.03±2.28（p=0.056）。电生理检查进一步显示，相较于非难治性CIDP患者，难治性CIDP患者的轴索损伤更突出（尺神经复合肌肉动作电位（ulnar CMAP）波幅分别为4.15±2.0与5.94±2.77 mV，p=0.011），脱髓鞘改变更严重：尺神经远端潜伏期分别为5.56±2.86与4.18±3.71 ms（p=0.008），正中神经远端潜伏期分别为7.94±5.62与6.52±6.64 ms（p=0.035），正中神经传导速度分别为30.21±12.59与37.48±12.44 m/s（p=0.035），正中神经F波潜伏期分别为58.66±25.73与42.30±13.77 ms（p=0.033）。此外，病程时长被证实为难治性CIDP的独立危险因素（p<0.05，95%置信区间[0.007, 0.076]）。 【结论】本研究全面系统地阐述了难治性CIDP的临床特征、临床病程分型、电生理特点及预后相关因素，清晰阐明了该亚型的多维度特征。本研究结果有助于深化学界对这一极具挑战性的CIDP亚型的认知，可为其临床管理与治疗策略制定提供参考依据。