期刊
JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM
卷 26, 期 3, 页码 242-268出版社
WILEY
DOI: 10.1111/jns.12455
关键词
CIDP; diagnosis; GRADE; guideline; treatment
资金
- European Academy of Neurology (EAN)
- GAIN Charity UK
- GBS/CIDP Foundation International
- Peripheral Nerve Society (PNS)
This study revised the consensus guideline on chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), distinguished typical CIDP and its variants, and reduced the levels of diagnostic certainty. The formulation of Good Practice Points provided guidance for the diagnosis and treatment of CIDP.
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).
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