Evaluation and treatment of refractory chronic inflammatory demyelinating polyradiculoneuropathy Article Swipe
Belinda Cruse
,
Lynette Kiers
·
YOU?
·
· 2025
· Open Access
·
· DOI: https://doi.org/10.1136/bmjno-2025-001318
YOU?
·
· 2025
· Open Access
·
· DOI: https://doi.org/10.1136/bmjno-2025-001318
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an inflammatory, immune-mediated neuropathy of the peripheral nerves and nerve roots. CIDP is unlikely to be a discrete disease entity, but rather a spectrum of related conditions, in which cell-mediated and humoral mechanisms act synergistically to cause damage to peripheral nerves. The 2021 guideline of the European Academy of Neurology/Peripheral Nerve Society on the diagnosis and treatment of CIDP has modified the CIDP spectrum to include typical CIDP and four well-defined CIDP variants. Patients with CIDP usually respond well to immunoglobulin therapy, steroids or plasmapheresis; however, 20–30% do not respond well, and approximately 15% remain refractory to all treatment modalities. Rituximab, mycophenolate mofetil and cyclophosphamide are of therapeutic benefit for some of these patients. Patients with some CIDP variants respond less well to immunotherapy, suggesting a difference in the pathogenic mechanisms underlying these variants. Potential novel treatments trialled in CIDP, targeting functionally relevant disease mechanisms, include neonatal Fc receptor blockers and complement inhibitors. These new treatment approaches are needed to optimise disease outcomes in refractory patients, and as an alternative for patients with suboptimal response requiring high doses or experiencing side effects from first-line therapies. Increasing the therapeutic options for patients with CIDP, particularly for refractory patients, highlights the need for more accurate diagnosis of typical CIDP and CIDP variants, objective evidence of treatment response and the need for reliable clinical biomarkers.
Related Topics
Concepts
Medicine
Polyradiculoneuropathy
Refractory (planetary science)
Peripheral neuropathy
Disease
Guideline
Cyclophosphamide
Chronic inflammatory demyelinating polyneuropathy
Intravenous Immunoglobulins
Internal medicine
Immunology
Clinical trial
Antibody
Salvage therapy
Gastroenterology
Intensive care medicine
Surgery
Guillain-Barre syndrome
Peripheral nerve
Hyporeflexia
Complete response
Dosing
Metadata
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.1136/bmjno-2025-001318
- https://neurologyopen.bmj.com/content/7/2/e001318.full.pdf
- OA Status
- diamond
- References
- 61
- OpenAlex ID
- https://openalex.org/W7105695662
All OpenAlex metadata
Raw OpenAlex JSON
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https://openalex.org/W7105695662Canonical identifier for this work in OpenAlex
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https://doi.org/10.1136/bmjno-2025-001318Digital Object Identifier
- Title
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Evaluation and treatment of refractory chronic inflammatory demyelinating polyradiculoneuropathyWork title
- Type
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articleOpenAlex work type
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enPrimary language
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2025Year of publication
- Publication date
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2025-07-01Full publication date if available
- Authors
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Belinda Cruse, Lynette KiersList of authors in order
- Landing page
-
https://doi.org/10.1136/bmjno-2025-001318Publisher landing page
- PDF URL
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https://neurologyopen.bmj.com/content/7/2/e001318.full.pdfDirect link to full text PDF
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YesWhether a free full text is available
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diamondOpen access status per OpenAlex
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https://neurologyopen.bmj.com/content/7/2/e001318.full.pdfDirect OA link when available
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Medicine, Polyradiculoneuropathy, Refractory (planetary science), Peripheral neuropathy, Disease, Guideline, Cyclophosphamide, Chronic inflammatory demyelinating polyneuropathy, Intravenous Immunoglobulins, Internal medicine, Immunology, Clinical trial, Antibody, Salvage therapy, Gastroenterology, Intensive care medicine, Surgery, Guillain-Barre syndrome, Peripheral nerve, Hyporeflexia, Complete response, DosingTop concepts (fields/topics) attached by OpenAlex
- Cited by
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0Total citation count in OpenAlex
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61Number of works referenced by this work
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