Pathophysiology and Emerging Therapies in Chronic Inflammatory Demyelinating Polyneuropathy
Abstract
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous immune-mediated neuropathy characterized by progressive or relapsing sensorimotor dysfunction. Recent advances have substantially improved our understanding of the pathophysiology of CIDP. Classical macrophage-mediated demyelination remains central to typical CIDP, whereas autoimmune nodopathies associated with IgG4 antibodies against nodal/paranodal proteins, such as neurofascin 155, contactin 1, and Caspr1, are now recognized as distinct disorders characterized by paranodal dysfunctions. Current therapies, including corticosteroids, immunoglobulins, and plasma exchange, remain effective for many patients; however, chronic treatment dependency and the lack of reliable biomarkers are important challenges. Recent advances in targeted therapies, particularly the introduction of neonatal Fc receptor (FcRn) inhibitors, have transformed the therapeutic landscape, while complement-targeted therapies remain under clinical investigation. This review summarizes the current understanding of CIDP immunopathology and discusses evolving therapeutic strategies, including FcRn-targeted therapies, for mechanism-based precision medicine.
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BibTeX
@article{yoshikawa2026pathophysiology,
title = {{Pathophysiology and Emerging Therapies in Chronic Inflammatory Demyelinating Polyneuropathy}},
author = {Masaaki Yoshikawa and Toshihiro Ide and Haruki Koike},
journal = {Internal Medicine},
year = {2026},
month = sep,
doi = {10.2169/internalmedicine.8132-26},
url = {https://www.jstage.jst.go.jp/article/internalmedicine/advpub/0/advpub_8132-26/_article},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Pathophysiology and Emerging Therapies in Chronic Inflammatory Demyelinating Polyneuropathy
AU - Masaaki Yoshikawa
AU - Toshihiro Ide
AU - Haruki Koike
PY - 2026
DA - 2026/09/15
JO - Internal Medicine
DO - 10.2169/internalmedicine.8132-26
UR - https://www.jstage.jst.go.jp/article/internalmedicine/advpub/0/advpub_8132-26/_article
AB - Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous immune-mediated neuropathy characterized by progressive or relapsing sensorimotor dysfunction. Recent advances have substantially improved our understanding of the pathophysiology of CIDP. Classical macrophage-mediated demyelination remains central to typical CIDP, whereas autoimmune nodopathies associated with IgG4 antibodies against nodal/paranodal proteins, such as neurofascin 155, contactin 1, and Caspr1, are now recognized as distinct disorders characterized by paranodal dysfunctions. Current therapies, including corticosteroids, immunoglobulins, and plasma exchange, remain effective for many patients; however, chronic treatment dependency and the lack of reliable biomarkers are important challenges. Recent advances in targeted therapies, particularly the introduction of neonatal Fc receptor (FcRn) inhibitors, have transformed the therapeutic landscape, while complement-targeted therapies remain under clinical investigation. This review summarizes the current understanding of CIDP immunopathology and discusses evolving therapeutic strategies, including FcRn-targeted therapies, for mechanism-based precision medicine.
SN - 1349-7235
SN - 0918-2918
ER -