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Immune-mediated neuropathies

Nature Reviews Disease Primers volume 4, Article number: 31 (2018) Cite this article

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Abstract

Since the discovery of an acute monophasic paralysis, later coined Guillain–Barré syndrome, almost 100 years ago, and the discovery of chronic, steroid-responsive polyneuropathy 50 years ago, the spectrum of immune-mediated polyneuropathies has broadened, with various subtypes continuing to be identified, including chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN). In general, these disorders are speculated to be caused by autoimmunity to proteins located at the node of Ranvier or components of myelin of peripheral nerves, although disease-associated autoantibodies have not been identified for all disorders. Owing to the numerous subtypes of the immune-mediated neuropathies, making the right diagnosis in daily clinical practice is complicated. Moreover, treating these disorders, particularly their chronic variants, such as CIDP and MMN, poses a challenge. In general, management of these disorders includes immunotherapies, such as corticosteroids, intravenous immunoglobulin or plasma exchange. Improvements in clinical criteria and the emergence of more disease-specific immunotherapies should broaden the therapeutic options for these disabling diseases.

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Fig. 1: Pathomechanisms of immune-mediated neuropathies.
Fig. 2: Structure of glycolipids to which antibody responses have been detected in immune-mediated neuropathies.
Fig. 3: The potential role of autoantibodies in immune-mediated neuropathies.
Fig. 4: Diagnostic algorithm.

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Reviewer information

Nature Reviews Disease Primers thanks G. Lauria, E. Nobile-Orazio, A. Uncini, J. Vallat and the other anonymous reviewer(s) for their contribution to the peer review of this work.

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Authors and Affiliations

  1. Department of Neurology, Medical Faculty, Heinrich Heine University, Düsseldorf, Germany

    Bernd C. Kieseier & Hans-Peter Hartung

  2. Neuroinflammation Laboratory, Brain and Mind Centre, University of Sydney, Sydney, Australia

    Emily K. Mathey

  3. Department of Neurology, University of Würzburg, Würzburg, Germany

    Claudia Sommer

Authors
  1. Bernd C. Kieseier
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  2. Emily K. Mathey
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  3. Claudia Sommer
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  4. Hans-Peter Hartung
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Contributions

Introduction (B.C.K. and H.-P.H.); Epidemiology (B.C.K., E.K.M. and C.S.); Mechanisms/pathophysiology (B.C.K., E.K.M. and H.-P.H.); Diagnosis, screening and prevention (B.C.K. and C.S.); Management (B.C.K., E.K.M., C.S. and H.-P.H.); Quality of life (B.C.K.); Outlook (B.C.K. and H.-P.H.); Overview of Primer (B.C.K.).

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Correspondence to Bernd C. Kieseier.

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Competing interests

B.C.K. reports receiving personal fees for consulting for Grifols and UCB. E.K.M. reports receiving funding from the GBS/CIDP Foundation International and the National Health and Medical Research Council of Australia and is a member of the Scientific Committee of the Trish Multiple Sclerosis Research Foundation. C.S. reports receiving personal fees for educational talks for Alnylam, CSL Behring, Grifols, Kedrion, Sanofi-Genzyme, Shire and Pfizer; personal fees for consulting for Air Liquide, Alnylam, Astellas, Baxalta and UCB; and research support to the institution from Kedrion. H.-P.H. reports CSL Behring fees for serving on a steering committee and from UCB for participating on advisory boards.

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Kieseier, B.C., Mathey, E.K., Sommer, C. et al. Immune-mediated neuropathies. Nat Rev Dis Primers 4, 31 (2018). https://doi.org/10.1038/s41572-018-0027-2

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