Disease Activity in Chronic Inflammatory Demyelinating Polyneuropathy: Association Between Circulating B Cell Subsets, Cytokine Levels and Clinical Outcomes.

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. 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. 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). 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.

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