Insights into refractory chronic inflammatory demyelinating polyneuropathy: a comprehensive real-world study

• DOI: 10.3389/fneur.2024.1326874
• Publication Date: 2024-01-31T12:12:00Z
• Authors: Yongsheng Zheng; Jianian Hu; Chong Sun; Kai Qiao; Yanyin Zhao; Bingyou Liu; Jian Sun; Jianying Xi; Sushan Luo; Jiahong Lu; Chongbo Zhao; Jie Lin
• Keywords: Refractory (planetary science)

Background Refractory 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 its clinical profile, electrophysiological characteristics potential risk factors associated with refractoriness remains be further elucidated. Methods Data in this cross-sectional study was collected reviewed from the Huashan Peripheral Neuropathy Database (HSPN). Included patients were categorized into refractory CIDP non-refractory groups based on treatment response. The compared between groups. Potential explored multivariate logistic regression model. Results Fifty-eight included. Four disease course patterns are described: relapsing–remitting form, stable secondary progressive form primary form. Compared patients, exhibited longer duration (48.96 ± 33.72 vs. 28.33 13.72 months, p = 0.038) worse functional impairment (MRC sum score, 46.08 12.69 52.81 7.34, 0.018; mRS, 2.76 0.93 2.33 0.99, 0.082; INCAT, 3.68 1.76 3.03 2.28, 0.056, respectively). Electrophysiological studies revealed greater axonal (4.15 2.0 5.94 2.77 mv, 0.011, ulnar CMAP) more severe demyelination (5.56 2.86 4.18 3.71 ms, 0.008, distal latency, 7.94 5.62 6.52 6.64 0.035, median latency; 30.21 12.59 37.48 12.44 m/s, conduction velocity; 58.66 25.73 42.30 13.77 0.033, F-wave latency), Disease shown an independent factor for ( < 0.05, 95%CI [0.007, 0.076]). Conclusion This provided description CIDP, addressing features, classification course, characteristics, prognostic factors, effectively elucidating various aspects. These findings contribute better might informative management strategies.