Chronic Inflammatory Demyelinating Polyneuropathy and Variants
- PMID: 41161995
- DOI: 10.1016/j.ncl.2025.05.015
Chronic Inflammatory Demyelinating Polyneuropathy and Variants
Abstract
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the prototypic chronic inflammatory polyneuropathy, characterized by well-defined clinical and electrodiagnostic criteria, despite the absence of biomarkers or full understanding of disease pathogenesis. It has both a typical form and 5 variants. IVIG remains the treatment of choice for induction or maintenance of remission, although corticosteroids, plasma exchange, and most recently, efgartigimod, demonstrate therapeutic efficacy. Subcutaneous immunoglobulin is indicated only as maintenance therapy. Validated outcome measures exist to track disability, strength, and quality of life and should be routinely employed in clinical practice.
Keywords: CIDP; CIDP treatment; CIDP variants; Electrodiagnostic demyelinating criteria; Multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy.
Copyright © 2025 Elsevier Inc. All rights reserved.
Conflict of interest statement
Disclosure A.M. Stino received consultancy fees for Argenx, Takeda, Sanofi, Merck, and CSL Behring, and has received research funding from the GBS-CIDP Foundation and Bristol Myers Squibb. J. Katz and R.J. Barohn have nothing relevant to disclose. M.M. Dimachkie received consultancy fees, payment or honoraria for lectures, presentations, article writing or educational events, and participated on a Data Safety Monitoring Board or Advisory Board for Abata/Third Rock, Abcuro, Amicus, ArgenX, Astellas, Cabaletta Bio, Catalyst, CNSA, Covance/Labcorp, CSL-Behring, Dianthus, Horizon, EMD Serono/Merck, Fortrea, Ig Society, Inc, Ipsen, Janssen, Medlink, Nuvig, Octapharma, Sanofi Genzyme, Shire Takeda, and TACT/Treat NMD; M.M. Dimachkie has received grants or from Alexion, United States/AstraZeneca, Alnylam Pharmaceuticals, United States, Amicus, United States, Argenx, The Netherlands, Bristol-Myers Squibb, United States, Catalyst, United States, CSL Behring, United States, FDA/OOPD, GlaxoSmithKline, United Kingdom, Genentech, United States, Grifols, Spain, Mitsubishi Tanabe Pharma, Japan, MDA, NIH, Novartis, Switzerland, Octapharma, Orphazyme, Ra Pharma/UCB Biopharma, Sanofi Genzyme, United States, Sarepta Therapeutics, United States, Shire Takeda, Spark Therapeutics, United States, and The Myositis Association, United States; M.M. Dimachkie received royalty fees for article writing from Wolters Kluwer Health/UpToDate.
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