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 - 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
UR - http://www.scopus.com/inward/record.url?scp=85111487398&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85111487398&partnerID=8YFLogxK
U2 - 10.1111/ene.14959
DO - 10.1111/ene.14959
M3 - Article
C2 - 34327760
AN - SCOPUS:85111487398
SN - 1351-5101
VL - 28
SP - 3556
EP - 3583
JO - European Journal of Neurology
JF - European Journal of Neurology
IS - 11
ER -