Short Communication| Volume 330, P23-27, May 15, 2019

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IVIG efficacy in CIDP patients is not associated with terminal complement inhibition

  • Christian W. Keller
    Department of Neurology with Institute of Translational Neurology, University Hospital Münster, Münster 48149, Germany

    Institute of Experimental Immunology, Department of Neuroinflammation, University of Zurich, Zürich 8057, Switzerland
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  • Isaak Quast
    Department of Immunology and Pathology, Central Clinical School, Monash University, Melbourne, Australia
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  • Marinos C. Dalakas
    Department of Neurology, Thomas Jefferson University, Philadelphia, USA

    Neuroimmunology Unit, Department of Pathophysiology, Faculty of Medicine, National and Kapodistrian University of Athens, Athens, Greece
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  • Jan D. Lünemann
    Corresponding author at: Department of Neurology with Institute of Translational Neurology, University Hospital Münster, Münster 48149, Germany.
    Department of Neurology with Institute of Translational Neurology, University Hospital Münster, Münster 48149, Germany

    Institute of Experimental Immunology, Department of Neuroinflammation, University of Zurich, Zürich 8057, Switzerland
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Published:February 09, 2019DOI:


      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.

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