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  • Review Article
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Advances in the diagnosis, pathogenesis and treatment of CIDP

Nature Reviews Neurology volume 7, pages 507–517 (2011)Cite this article

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Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common chronic autoimmune neuropathy. Despite clinical challenges in diagnosis—owing in part to the existence of disease variants, and different views on how many electrophysiological abnormalities are needed to document demyelination—consensus criteria seem to have been reached for research or clinical practice. Current standard of care involves corticosteroids, intravenous immunoglobulin (IVIg) and/or plasmapheresis, which provide short-term benefits. Maintenance therapy with IVIg can induce sustained remission, increase quality of life and prevent further axonal loss, but caution is needed to avoid overtreatment. Commonly used immunosuppressive drugs offer minimal benefit, necessitating the development of new therapies for treatment-refractory patients. Advances in our understanding of the underlying immunopathology in CIDP have identified new targets for future therapeutic efforts, including T cells, B cells, and transmigration and transduction molecules. New biomarkers and scoring systems represent emerging tools with the potential to predict therapeutic responses and identify patients with active disease for enrollment into clinical trials. This Review highlights the recent advances in diagnosing CIDP, provides an update on the immunopathology including new target antigens, and discusses current treatments, ongoing challenges and future therapeutic directions.

Key Points

  • Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common acquired chronic autoimmune neuropathy

  • Despite disease heterogeneity, recently revised diagnostic criteria provide an optimal balance between sensitivity and specificity

  • Molecules within the non-compact myelin and points of Schwann cell–axon interaction, rather than within compact myelin, seem to be the putative target antigens

  • Corticosteroids, intravenous immunoglobulin (IVIg) and plasmapheresis provide short-term benefits; IVIg is used for long-term maintenance

  • Potential new therapeutic approaches involve targeting key factors in the immunopathogenesis of CIDP, including T cells, B cells and complement

  • Progress in clinical trial design is focused on clinically meaningful tools to define therapeutic responses, enrolling only patients with active disease and exploring biomarkers that predict response to therapies

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Figure 1
Figure 2: Scheme depicting the main immunopathogenic network involved in CIDP.

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Acknowledgements

L. Barclay, freelance writer and reviewer, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape, LLC-accredited continuing medical education activity associated with this article.

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  1. Department of Pathophysiology, Neuroimmunology Unit, National University of Athens Medical School, Building 16, Room 39, 75 Mikras Asias Street, Athens, 11527, Greece

    Marinos C. Dalakas

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The author has received speaking honoraria or consulting fees for serving on the steering committees of the following companies: Biogen, Novartis, Octapharma, Talecris/Grifol.

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Dalakas, M. Advances in the diagnosis, pathogenesis and treatment of CIDP. Nat Rev Neurol 7, 507–517 (2011). https://doi.org/10.1038/nrneurol.2011.121

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