Journal Article

DZNE-2026-00048

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Aberrant Complement Activation Is a Prominent Feature of Chronic Inflammatory Demyelinating Polyneuropathy.

Stascheit, F. ; Preßler, H.DZNE* ; Stein, K. ; Pernice, H. F. ; Hahn, K. ; Meisel, A. ; Lünemann, J. D.

2026
Wolters Kluwer Philadelphia, Pa.

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Please use a persistent id in citations: doi:10.1212/NXI.0000000000200542

Abstract: To comprehensively characterize complement pathway activation in chronic inflammatory demyelinating polyneuropathy (CIDP) and its association with clinical disease features using advanced complement profiling.Complement protein levels indicative of classical, lectin, and alternative pathway activation were quantified by multiplex ELISA and compared between 28 patients with typical CIDP, 24 patients with Charcot-Marie Tooth neuropathy (CMT), and 24 demographically matched healthy controls (HD).Serum levels of activated complement proteins-C3a, C4a, Ba, Bb, C5a, and the soluble terminal complement complex sC5b-9 (sTCC)-were significantly elevated in CIDP patients compared to healthy donors (HD) (p < 0.001). Except for C3a, these protein levels were also significantly higher in CIDP patients than in those with Charcot-Marie-Tooth disease (CMT). Among CIDP patients, those with active, unstable disease exhibited significantly higher levels of terminal complement components (C5a and sTCC) compared to patients with stable disease or in remission (p < 0.001).These findings highlight the critical involvement of aberrant complement activation in the pathophysiology of CIDP and provide a rationale for further investigation into targeted complement inhibition as a therapeutic approach.

Keyword(s): Humans (MeSH) ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating: blood (MeSH) ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating: immunology (MeSH) ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating: physiopathology (MeSH) ; Male (MeSH) ; Female (MeSH) ; Middle Aged (MeSH) ; Complement Activation: physiology (MeSH) ; Adult (MeSH) ; Charcot-Marie-Tooth Disease: blood (MeSH) ; Charcot-Marie-Tooth Disease: immunology (MeSH) ; Aged (MeSH)

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Contributing Institute(s):

1. Autoimmune Encephalopathies (AG Prüß)

Research Program(s):

1. 353 - Clinical and Health Care Research (POF4-353) (POF4-353)

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