Objective: To revise the 2010 consensus guideline on chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods: 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). Results: 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).
Bibliographical noteFunding Information:
The CIDP Guideline Task Force is grateful to the European Academy of Neurology (EAN), the Peripheral Nerve Society (PNS), the GBS/CIDP Foundation International and the GAIN Charity UK for granting unrestricted financial support. Date of EAN approval: May 14, 2021.
P. Y. K. Van den B. reports honoraria, consultancy fees, advisory boards from Pfizer, Genzyme, CSL Behring, LFB, Natus, UCB Pharma, Alnylam Pharmaceuticals. P. A. van D. reports grants from Baxalta, Sanquin, Prinses Beatrix Spierfonds, Talecris. R. D. M. H. reports honoraria, consultancy, travel expenses, and departmental donations from Alnylam, Argenx, Grifols, and CSL Behring. B. A. has nothing to disclose. P. V. fees for consultancy and advisory boards (all to department) from: Annexon, Argenx, Hansa, Immunic, Octapharma, Roche. J. A. A. reports grants from CSL Behring and consultancy from CSL Behring, Grifols, Biopharma, Biotest. S. A. reports grants and honoraria from LFB, Pfizer, CSL Behring. P. H. P. H. B. has nothing to disclose. D. R. C. reports consultancy fees from Acetylon Pharmaceuticals, Alnylam Pharmaceuticals, Annexon Biosciences, Akros Pharma, Biotest Pharmaceuticals, Boehringer Ingelheim, Cigna Health Management, CSL Behring, DP Clinical, Grifols, Karos Pharmaceuticals, Neurocrine Biosciences, Novartis, Octapharma, Pharnext, Sun Pharmaceuticals, Syntimmune, and stock options from Syntimmune. F. E. reports grants from Prinses Beatrix Spierfonds, ZonMw, and consultancy from Astellas, UCB Pharma. H. S. G. reports grants from Prinses Beatrix Spierfonds. T. H. has nothing to disclose. S. K. reports Kakenhi grants from the Ministry of Welfare in Japan. R. A. L. reports consultancy fees from CSL Behring, Grifols, Optioncare, Kedrion, Biotest, Axelacare, Pharnext. M. P. L. reports consultancy fees from Octapharma, CSL Behring and a patent for antiganglioside antibodies licenced to Fujierbo (Johns Hopkins Hospital). E. N.‐O. reports consulting fee, honorarium, and advisory/scientific boards from Argenx, Belgium; Astellas, the Netherlands; Baxter/Takeda, Italy, and United States; CSL Behring, Italy, and Switzerland; Grifols, Spain; Janssen, united States; Kedrion, Italy; LFB, France; Novartis, Switzerland; Roche, Switzerland; Sanofi, united States; UCB,UK. L. Q. reports grants and consultancy fees from Grifols, Novartis. Y. A. R. reports grants from LFB and consultancy fees from LFB, CSL Behring. C. S. reports grants from Kedrion, consultancy fees from Airliquidw, Alnylam Pharmaceuticals. H. A. T. reports grants from EU‐Tubitak.
© 2021 European Academy of Neurology and Peripheral Nerve Society
© 2021 European Academy of Neurology and Peripheral Nerve Society.