title: Terminal complement activation is increased and associated with disease severity in CIDP.
abstract: 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.
authors.affiliation: Department of Neuroinflammation Institute of Experimental Immunology University of Zürich Zürich Switzerland.
authors.forename: Isaak
authors.initials: I
authors.lastname: Quast
authors.affiliation: Department of Neuroinflammation Institute of Experimental Immunology University of Zürich Zürich Switzerland.
authors.forename: Christian W
authors.initials: CW
authors.lastname: Keller
authors.affiliation: Department of Rheumatology and Clinical Immunology Charité-University Medicine Berlin Berlin Germany.
authors.forename: Falk
authors.initials: F
authors.lastname: Hiepe
authors.affiliation: Department of Neurology Philipps-University Marburg Germany.
authors.forename: Björn
authors.initials: B
authors.lastname: Tackenberg
authors.affiliation: Department of Neuroinflammation Institute of Experimental Immunology University of Zürich Zürich Switzerland; Department of Neurology University Hospital Basel Basel Switzerland.
authors.forename: Jan D
authors.initials: JD
authors.lastname: Lünemann