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

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

doi:10.1111/ene.14959

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

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

Neurology

Research output: Contribution to journal › Article › peer-review

218 Scopus citations

Abstract

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).

Original language	English (US)
Pages (from-to)	3556-3583
Number of pages	28
Journal	European Journal of Neurology
Volume	28
Issue number	11
Early online date	Jul 30 2021
DOIs	https://doi.org/10.1111/ene.14959
State	Published - Nov 2021

Bibliographical note

Funding 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.

Funding Information:
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.

Publisher Copyright:
© 2021 European Academy of Neurology and Peripheral Nerve Society

Keywords

CIDP
GRADE
diagnosis
guideline
treatment

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1111/ene.14959

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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. European Journal of Neurology, 28(11), 3556-3583. https://doi.org/10.1111/ene.14959

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. / Van den Bergh, Peter Y.K.; van Doorn, Pieter A.; Hadden, Robert D.M. et al.
In: European Journal of Neurology, Vol. 28, No. 11, 11.2021, p. 3556-3583.

Research output: Contribution to journal › Article › peer-review

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 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', European Journal of Neurology, vol. 28, no. 11, pp. 3556-3583. https://doi.org/10.1111/ene.14959

Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA 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. European Journal of Neurology. 2021 Nov;28(11):3556-3583. Epub 2021 Jul 30. doi: 10.1111/ene.14959

@article{c25881278bb94cc9a9243516200262d8,

title = "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",

abstract = "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).",

keywords = "CIDP, GRADE, diagnosis, guideline, treatment",

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

note = "Funding 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. Funding Information: 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. Publisher Copyright: {\textcopyright} 2021 European Academy of Neurology and Peripheral Nerve Society",

year = "2021",

month = nov,

doi = "10.1111/ene.14959",

language = "English (US)",

volume = "28",

pages = "3556--3583",

journal = "European Journal of Neurology",

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TY - JOUR

T1 - European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy

T2 - Report of a joint Task Force—Second revision

AU - Van den Bergh, Peter Y.K.

AU - van Doorn, Pieter A.

AU - Hadden, Robert D.M.

AU - Avau, Bert

AU - Vankrunkelsven, Patrik

AU - Allen, Jeffrey A.

AU - Attarian, Shahram

AU - Blomkwist-Markens, Patricia H.

AU - Cornblath, David R.

AU - Eftimov, Filip

AU - Goedee, H. Stephan

AU - Harbo, Thomas

AU - Kuwabara, Satoshi

AU - Lewis, Richard A.

AU - Lunn, Michael P.

AU - Nobile-Orazio, Eduardo

AU - Querol, Luis

AU - Rajabally, Yusuf A.

AU - Sommer, Claudia

AU - Topaloglu, Haluk A.

N1 - Funding 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. Funding Information: 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. Publisher Copyright: © 2021 European Academy of Neurology and Peripheral Nerve Society

PY - 2021/11

Y1 - 2021/11

N2 - 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).

AB - 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).

KW - CIDP

KW - GRADE

KW - diagnosis

KW - guideline

KW - treatment

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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

Abstract

Bibliographical note

Keywords

UN SDGs

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