Article Text
Abstract
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a heterogeneous but clinically well-described disease within circumscribed parameters. It is immunologically mediated through several poorly understood mechanisms. First-line therapies with steroids, intravenous immunoglobulin (IVIG) or plasma exchange are each effective in about two-thirds of patients. These treatments are seldom associated with complete resolution or cure, and often pose considerable practical, financial and medical implications.
Our understanding of many of the key pathological processes in autoimmune diseases is expanding, and novel targeted therapeutics are being developed with promise in several autoimmune neurological disorders.
This narrative review looks first at detailing key pathogenic mechanisms of disease in CIDP, followed by an in-depth description of potential novel therapies and the current evidence of their application in clinical practice.
- IMMUNOLOGY
- NEUROIMMUNOLOGY
- NEUROMUSCULAR
- NEUROPATHY
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Footnotes
X @mike_the_nerve
Contributors DM and HM were responsible for the literature search and writing of the publication. FE and MPL were responsible for edits and reviews. SK was responsible for the concept, design, edits and reviews.
Funding Professor L is supported by the National Institute for Health Research, University College London Hospitals Biomedical Research Centre
Competing interests FE has received funding in grants from CSL Behring, Grifols, Terumo BCT, Takeda, Kendion and the GBS/CIDP foundation. He has also received consultation fees (paid to institution) from Takeda, Dianthus, Sanofi and Argenx. MPL has given advice on ad hoc advisory boards particularly on trial design to Roche, AstraZeneca, Sanofi, UCB, Sanofi, Takeda, Polyneuron and BeiGene (conference expenses and advisory board). Unrestricted conference expenses have been received from Beigene and CSL Behring. He has received research grants from Charitable Foundations: Patrick Berthoud Charitable Trust, ABN, Guarantors of Brain, National Brain Appeal, UCLH Charities, Leonard Wolfson Foundation, Medical Research Council, GBS CIDP Foundation International, New Zealand Medical Foundation, GAIN UK. SK has received conference expenses from CSL Behring.
Provenance and peer review Not commissioned; externally peer reviewed.