Chronic inflammatory demyelinating polyneuropathy From immunology and biomarkers to treatment

This thesis, Chronic Inflammatory Demyelinating Polyneuropathy: From Immunology and Biomarkers to Treatment, explores the immunopathological mechanisms underlying Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) and the search for reliable biomarkers to monitor disease activity and guide therapy. First, potential viral triggers were investigated using VIDISCA-NGS sequencing of serum and cerebrospinal fluid from CIDP patients. No significant differences in viral profiles were observed compared to controls, suggesting that persistent viral infection is unlikely to sustain the disease, although an initial viral trigger cannot be excluded. Next, B- and T-cell receptor repertoire analyses were performed to evaluate their value as biomarkers. While some patients showed highly expanded lymphocyte clones, overall repertoire diversity and clonality did not differ significantly between patients and controls, indicating limited diagnostic or prognostic utility at the whole-repertoire level.
Further, the identification of unique T-cell clones in nerve tissue but not in peripheral blood highlights a localized immune response that may contribute to CIDP pathogenesis. Serum neurofilament light chain (sNfL) was also evaluated as a marker of axonal damage and disease activity. Elevated sNfL correlated with axonal injury and treatment response in a subset of patients, suggesting potential value for monitoring specific disease courses.
Finally, corticosteroid treatment outcomes were analyzed across different regimens, showing similar efficacy with a 60% overall response rate and a 33% five-year remission probability. Short-pulsed regimens may minimize long-term side effects without compromising efficacy. Overall, this thesis advances understanding of CIDP immunopathology, biomarker development, and treatment optimization.