Abstract
Patients with acute and chronic inflammatory demyelinating neuropathies exhibit elevated
serum and cerebrospinal fluid (CSF) levels of terminal complement activation products
and therapeutic inhibition of complement activation is currently tested for its safety
and efficacy in patients with Guillain-Barré syndrome (GBS). Here, we determined serum
levels of the complement activation products C3a, C5a and the soluble terminal complement
complex (sTCC) in 39 individuals with chronic inflammatory demyelinating polyneuropathy
(CIDP) who participated in one of the largest ever conducted clinical trial in patients
with CIDP (ICE trial) and received Intravenous Immunoglobulin (IVIG) or placebo (albumin)
in 3 week intervals for up to 24 weeks. In placebo-treated patients with spontaneous
disease remission, serum sTCC levels moderately decreased over time. Levels of complement
activation products were, however, not modulated by IVIG and remained unchanged in
patients with a beneficial response to IVIG therapy as compared to those with steady
or worsened disease. These results suggest that the therapeutic efficacy of IVIG in
CIDP is based on immunomodulatory mechanisms different from complement inhibition.
Terminal complement activation merits further investigation as a surrogate marker
for disease progression and therapeutic target in patients with CIDP.
Graphical abstract
Graphical Abstract
Keywords
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Article info
Publication history
Published online: February 09, 2019
Accepted:
February 7,
2019
Received in revised form:
February 5,
2019
Received:
November 9,
2018
Identification
Copyright
© 2019 Elsevier B.V. All rights reserved.
