Open AccessTerminal complement activation is increased and associated with disease severity in CIDP
Author(s) -
Quast Isaak,
Keller Christian W.,
Hiepe Falk,
Tackenberg Björn,
Lünemann Jan D.
Publication year - 2016
Publication title -
annals of clinical and translational neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.824
H-Index - 42
ISSN - 2328-9503
DOI - 10.1002/acn3.331
Subject(s) - medicine , complement system , pathogenesis , immunology , anaphylatoxin , chronic inflammatory demyelinating polyneuropathy , plasmapheresis , complement (music) , peripheral neuropathy , antibody , diabetes mellitus , endocrinology , biochemistry , chemistry , complementation , gene , phenotype
Chronic inflammatory demyelinating polyneuropathy ( CIDP ) is the most common chronic autoimmune neuropathy. While both cell‐mediated and humoral mechanisms contribute to its pathogenesis, the rapid clinical response to plasmapheresis implicates a circulating factor responsible for peripheral nerve injury. We report that treatment‐naïve patients with CIDP show increased serum and CSF levels of the anaphylatoxin C5a and the soluble terminal complement complex ( sTCC ). Systemic terminal complement activation correlates with clinical disease severity as determined by the Inflammatory Neuropathy Cause and Treatment ( INCAT ) disability scale. These data indicate that complement activation contributes to peripheral nerve injury and suggest that complement inhibition should be explored for its potential therapeutic merit in CIDP .