Nerve-Muscle Unit, Neurology Service, Department of Clinical Neurosciences, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland..
J Neurol Sci. 2024 Nov 15;466:123259. doi: 10.1016/j.jns.2024.123259. Epub 2024 Sep 25.
To describe the clinical characteristics and long term outcome of CIDP patients according to 2021 EAN/PNS diagnostic certainty categories.
We reviewed clinical data, response to treatment, cerebrospinal fluid examination, and nerve conduction studies parameters of 39 adult "CIDP" and 24 "possible CIDP" patients. Data were collected at diagnosis and after one (T1), two (T2), three (T3) and five years (T5).
At diagnosis, "possible CIDP" patients' phenotypes were more atypical (especially focal/multifocal, p < .01) and "CIDP" patients had a higher NIS and INCAT scores (p = .08 and 0.08). Compared to baseline: median NIS score decreased in "CIDP" and was stable in "possible CIDP" patients at T1 (p < .05), T2 (p < .05) and T3 (p < .01); median MRC score slightly increased in "CIDP" and was stable in "possible CIDP" patients at T2 (p < .05); and INCAT disability scale slightly decreased in "CIDP" and was stable in "possible CIDP" patients at T3 (p < .05). The proportion of moderate to severely disabled (mRS > 2) patients in "possible CIDP" group was higher than in "CIDP" group (not significant). "CIDP" patients had a better objective response to immunotherapy (59 % responders) than "possible CIDP" patients (29 % responders, p < .05), especially among typical CIDP patients (86 % of responders in "CIDP" versus 33 % of responders in "possible CIDP" patients, p < .05).
"CIDP" patients had a more severe neuropathy, estimated with the NIS and INCAT scores, and "possible CIDP" patients had a more atypical phenotype at baseline. Our data suggest that long-term patient outcome and response to immunotherapy is better in "CIDP" than "possible CIDP".
根据 2021 年 EAN/PNS 诊断确定性类别，描述 CIDP 患者的临床特征和长期预后。
我们回顾了 39 名成年“CIDP”和 24 名“可能 CIDP”患者的临床数据、治疗反应、脑脊液检查和神经传导研究参数。数据在诊断时以及 1 年（T1）、2 年（T2）、3 年（T3）和 5 年（T5）时收集。
在诊断时，“可能 CIDP”患者的表型更不典型（尤其是局灶性/多灶性，p<0.01），而“CIDP”患者的 NIS 和 INCAT 评分更高（p=0.08 和 0.08）。与基线相比：“CIDP”患者的中位 NIS 评分在 T1（p<0.05）、T2（p<0.05）和 T3（p<0.01）时下降，而“可能 CIDP”患者的评分保持稳定；“CIDP”患者的中位 MRC 评分略有增加，而“可能 CIDP”患者的评分在 T2 时保持稳定（p<0.05）；“CIDP”患者的 INCAT 残疾量表略有下降，而“可能 CIDP”患者的评分在 T3 时保持稳定（p<0.05）。“可能 CIDP”组中中度至重度残疾（mRS>2）患者的比例高于“CIDP”组（无显著差异）。“CIDP”患者对免疫治疗的客观反应更好（59%的应答者），而“可能 CIDP”患者（29%的应答者，p<0.05），尤其是在典型 CIDP 患者中（“CIDP”组的 86%应答者与“可能 CIDP”组的 33%应答者，p<0.05）。
“CIDP”患者的神经病变更严重，估计用 NIS 和 INCAT 评分来衡量，而“可能 CIDP”患者在基线时的表型更不典型。我们的数据表明，“CIDP”患者的长期预后和对免疫治疗的反应优于“可能 CIDP”患者。
