OBJECTIVE To evaluate the relationship between immunological markers and clinical outcome measures in a mixed cohort of patients with typical CIDP and CIDP variants at different disease stages. METHODS Twenty-three typical, 16 multifocal and 5 distal CIDP patients were included. Twenty-five sex and age-matched healthy controls and 12 patients with Charcot-Marie-Tooth type 1A (CMT1A) disease served as controls. Peripheral B-cell populations were analyzed by flow cytometry. IL6, IL10, TNFA mRNA and mir-21, mir-146a and mir-155-5p expression levels were evaluated by real-time polymerase chain reaction in Peripheral blood mononuclear cells (PBMC) and/or skin biopsy specimens. Results were then assessed for a possible association with clinical disability scores and intraepidermal nerve fiber densities (IENFD) in distal leg. RESULTS We detected significant reduction in naive B-cells (p≤0.001), plasma cells (p≤0.001) and regulatory B-cells (p<0.05), and an elevation in switched memory B-cells (p≤0.001) in CIDP compared to healthy controls. CMT1A and CIDP patients had comparable B-cell subset distribution. CIDP cases had significantly higher TNFA and IL10 gene expression levels in PBMC compared to healthy controls (p<0.05 and p≤0.01, respectively). IENFDs in distal leg showed a moderate negative correlation with switched memory B-cell ratios (r=-0.51, p<0.05) and a moderate positive correlation with plasma cell ratios (r=0.46, p<0.05). INCAT sum scores showed a moderate positive correlation with IL6 gene expression levels in PBMC (r=0.54, p<0.05). DISCUSSION Altered B-cell homeostasis and IL10 and TNFA gene expression levels imply chronic antigen exposure and overactivity in humoral immune system, and seem to be a common pathological pathway in both typical CIDP and CIDP variants.

中文翻译：

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慢性炎症性脱髓鞘性多发性神经病的疾病活动：循环 B 细胞亚群、细胞因子水平和临床结果之间的关联。

目的 评估不同疾病阶段具有典型 CIDP 和 CIDP 变异的混合患者队列中免疫学标志物与临床结果测量之间的关系。方法 纳入 23 名典型、16 名多灶性和 5 名远端 CIDP 患者。25 名性别和年龄匹配的健康对照者和 12 名患有腓骨肌萎缩症 1A 型 (CMT1A) 病的患者作为对照。通过流式细胞术分析外周 B 细胞群。通过实时聚合酶链反应评估外周血单核细胞 (PBMC) 和/或皮肤活检标本中的 IL6、IL10、TNFA mRNA 以及 mir-21、mir-146a 和 mir-155-5p 表达水平。然后评估结果与临床残疾评分和远端腿部表皮内神经纤维密度（IENFD）之间可能的关联。结果 我们检测到 CIDP 中初始 B 细胞 (p≤0.001)、浆细胞 (p≤0.001) 和调节性 B 细胞 (p<0.05) 显着减少，而转换记忆 B 细胞 (p≤0.001) 显着增加与健康对照相比。CMT1A 和 CIDP 患者具有相似的 B 细胞亚群分布。与健康对照相比，CIDP 病例的 PBMC 中 TNFA 和 IL10 基因表达水平显着较高（分别为 p<0.05 和 p≤0.01）。远端腿的 IENFD 与切换记忆 B 细胞比率呈中度负相关（r=-0.51，p<0.05），与浆细胞比率呈中度正相关（r=0.46，p<0.05）。INCAT 总分与 PBMC 中 IL6 基因表达水平呈中度正相关（r=0.54，p<0.05）。讨论 B 细胞稳态以及 IL10 和 TNFA 基因表达水平的改变意味着体液免疫系统的慢性抗原暴露和过度活跃，并且似乎是典型 CIDP 和 CIDP 变体的常见病理途径。