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Van den Bergh PYK, van Doorn PA, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision. J Peripher Nerv Syst. 2021;26(3):242-268doi: 10.1111/jns.12455.
Van den Bergh, P. Y. K., van Doorn, P. A., Hadden, R. D. M., Avau, B., Vankrunkelsven, P., Allen, J. A., Attarian, S., Blomkwist-Markens, P. H., Cornblath, D. R., Eftimov, F., Goedee, H. S., Harbo, T., Kuwabara, S., Lewis, R. A., Lunn, M. P., Nobile-Orazio, E., Querol, L., Rajabally, Y. A., Sommer, C., & Topaloglu, H. A. (2021). European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision. Journal of the peripheral nervous system : JPNS, 26(3), 242-268. https://doi.org/10.1111/jns.12455
Van den Bergh, Peter Y K, et al. "European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision." Journal of the peripheral nervous system : JPNS vol. 26,3 (2021): 242-268. doi: https://doi.org/10.1111/jns.12455
Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA, Attarian S, Blomkwist-Markens PH, Cornblath DR, Eftimov F, Goedee HS, Harbo T, Kuwabara S, Lewis RA, Lunn MP, Nobile-Orazio E, Querol L, Rajabally YA, Sommer C, Topaloglu HA. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision. J Peripher Nerv Syst. 2021 Sep;26(3):242-268. doi: 10.1111/jns.12455. Epub 2021 Jul 30. PMID: 34085743.
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J Peripher Nerv Syst. 2021 Sep;26(3):242-268. doi: 10.1111/jns.12455. Epub 2021 Jul 30.

European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision.

Journal of the peripheral nervous system : JPNS

Peter Y K Van den Bergh, Pieter A van Doorn, Robert D M Hadden, Bert Avau, Patrik Vankrunkelsven, Jeffrey A Allen, Shahram Attarian, Patricia H Blomkwist-Markens, David R Cornblath, Filip Eftimov, H Stephan Goedee, Thomas Harbo, Satoshi Kuwabara, Richard A Lewis, Michael P Lunn, Eduardo Nobile-Orazio, Luis Querol, Yusuf A Rajabally, Claudia Sommer, Haluk A Topaloglu

Affiliations

  1. Neuromuscular Reference Centre, Department of Neurology, University Hospital Saint-Luc, Brussels, Belgium.
  2. Department of Neurology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.
  3. Department of Neurology, King's College Hospital, London, UK.
  4. Cochrane Belgium, CEBAM, Leuven, Belgium and CEBaP, Belgian Red Cross, Mechelen, Belgium.
  5. Cochrane Belgium, CEBAM, Leuven, Belgium.
  6. Department of Neurology, University of Minnesota, Minneapolis, Minnesota, USA.
  7. Centre de Référence des Maladies Neuromusculaires et de la SLA, APHM, CHU Timone, Marseille, France.
  8. Patient Representative GBS/CIDP Foundation International, International Office, Philadelphia, Pennsylvania, USA.
  9. Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
  10. Department of Neurology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.
  11. Department of Neuromuscular Disorders, University Medical Centre Utrecht, Utrecht, The Netherlands.
  12. Department of Neurology, Århus University Hospital, Århus, Denmark.
  13. Department of Neurology, Chiba University Hospital, Chiba, Japan.
  14. Department of Neurology, Cedars-Sinai Medical Center, Los Angeles, California, USA.
  15. Department of Neurology and MRC Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery, London, UK.
  16. Neuromuscular and Neuroimmunology Service, IRCCS Humanitas Clinical and Research Center, Department of Medical Biotechnology and Translational Medicine, University of Milan, Milan, Italy.
  17. Neuromuscular Diseases Unit-Neurology Department, Hospital de la Santa Creu I Sant Pau, Barcelona, Spain.
  18. Regional Neuromuscular Service, Neurology, Queen Elizabeth Hospital Birmingham, Birmingham, UK.
  19. Neurology Clinic, University Hospital Würzburg, Würzburg, Germany.
  20. Department of Pediatrics, Yeditepe University, ?stanbul, Turkey.

PMID: 34085743 DOI: 10.1111/jns.12455

Abstract

To revise the 2010 consensus guideline on chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Seventeen disease experts, a patient representative, and two Cochrane methodologists constructed 12 Population/Intervention/Comparison/Outcome (PICO) questions regarding diagnosis and treatment to guide the literature search. Data were extracted and summarized in GRADE summary of findings (for treatment PICOs) or evidence tables (for diagnostic PICOs). Statements were prepared according to the GRADE Evidence-to-Decision frameworks. Typical CIDP and CIDP variants were distinguished. The previous term "atypical CIDP" was replaced by "CIDP variants" because these are well characterized entities (multifocal, focal, distal, motor, or sensory CIDP). The levels of diagnostic certainty were reduced from three (definite, probable, possible CIDP) to only two (CIDP and possible CIDP), because the diagnostic accuracy of criteria for probable and definite CIDP did not significantly differ. Good Practice Points were formulated for supportive criteria and investigations to be considered to diagnose CIDP. The principal treatment recommendations were: (a) intravenous immunoglobulin (IVIg) or corticosteroids are strongly recommended as initial treatment in typical CIDP and CIDP variants; (b) plasma exchange is strongly recommended if IVIg and corticosteroids are ineffective; (c) IVIg should be considered as first-line treatment in motor CIDP (Good Practice Point); (d) for maintenance treatment, IVIg, subcutaneous immunoglobulin or corticosteroids are recommended; (e) if the maintenance dose of any of these is high, consider either combination treatments or adding an immunosuppressant or immunomodulatory drug (Good Practice Point); and (f) if pain is present, consider drugs against neuropathic pain and multidisciplinary management (Good Practice Point).

© 2021 European Academy of Neurology and Peripheral Nerve Society.

Keywords: CIDP; GRADE; diagnosis; guideline; treatment

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