The Role of the Complement System in Chronic Inflammatory Demyelinating Polyneuropathy: Implications for Complement-Targeted Therapies

Luis A. Querol, Hans Peter Hartung, Richard A. Lewis, Pieter A. van Doorn, Timothy R. Hammond, Nazem Atassi, Miguel Alonso-Alonso, Marinos C. Dalakas*

*Corresponding author for this work

Research output: Contribution to journalReview articleAcademicpeer-review

19 Citations (Scopus)
33 Downloads (Pure)

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common, heterogeneous, immune-mediated neuropathy, characterized by predominant demyelination of motor and sensory nerves. CIDP follows a relapsing–remitting or a progressive course and causes substantial disability. The pathogenesis of CIDP involves a complex interplay of multiple aberrant immune responses, creating a pro-inflammatory environment, subsequently inflicting damage on the myelin sheath. Though the exact triggers are unclear, diverse immune mechanisms encompassing cellular and humoral pathways are implicated. The complement system appears to play a role in promoting macrophage-mediated demyelination. Complement deposition in sural nerve biopsies, as well as signs of increased complement activation in serum and CSF of patients with CIDP, suggest complement involvement in CIDP pathogenesis. Here, we present a comprehensive overview of the preclinical and clinical evidence supporting the potential role of the complement system in CIDP. This understanding furnishes a strong rationale for targeting the complement system to develop new therapies that could serve the unmet needs of patients affected by CIDP, particularly in those refractory to standard therapies.

Original languageEnglish
Pages (from-to)864-873
Number of pages10
JournalNeurotherapeutics
Volume19
Issue number3
DOIs
Publication statusPublished - 4 Apr 2022

Bibliographical note

Funding Information:
Editorial support was funded by Sanofi.

Publisher Copyright:
© 2022, The Author(s).

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