ADVANCES IN THE DIAGNOSIS PATHOGENESIS AND TREATMENT OF CIDP PDF

Chronic inflammatory demyelinating polyneuropathy CIDP is the most common chronic autoimmune neuropathy. Despite clinical challenges in diagnosis-owing in part to the existence of disease variants, and different views on how many electrophysiological abnormalities are needed to document demyelination-consensus criteria seem to have been reached for research or clinical practice. Maintenance therapy with IVIg can induce sustained remission, increase quality of life and prevent further axonal loss, but caution is needed to avoid overtreatment. Commonly used immunosuppressive drugs offer minimal benefit, necessitating the development of new therapies for treatment-refractory patients. Advances in our understanding of the underlying immunopathology in CIDP have identified new targets for future therapeutic efforts, including T cells, B cells, and transmigration and transduction molecules.

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A Nature Research Journal. Chronic inflammatory demyelinating polyneuropathy CIDP is the most common chronic autoimmune neuropathy. Despite clinical challenges in diagnosis—owing in part to the existence of disease variants, and different views on how many electrophysiological abnormalities are needed to document demyelination—consensus criteria seem to have been reached for research or clinical practice.

Maintenance therapy with IVIg can induce sustained remission, increase quality of life and prevent further axonal loss, but caution is needed to avoid overtreatment. Commonly used immunosuppressive drugs offer minimal benefit, necessitating the development of new therapies for treatment-refractory patients. Advances in our understanding of the underlying immunopathology in CIDP have identified new targets for future therapeutic efforts, including T cells, B cells, and transmigration and transduction molecules.

New biomarkers and scoring systems represent emerging tools with the potential to predict therapeutic responses and identify patients with active disease for enrollment into clinical trials. This Review highlights the recent advances in diagnosing CIDP, provides an update on the immunopathology including new target antigens, and discusses current treatments, ongoing challenges and future therapeutic directions.

Chronic inflammatory demyelinating polyneuropathy CIDP is the most common acquired chronic autoimmune neuropathy. Despite disease heterogeneity, recently revised diagnostic criteria provide an optimal balance between sensitivity and specificity. Molecules within the non-compact myelin and points of Schwann cell—axon interaction, rather than within compact myelin, seem to be the putative target antigens. Corticosteroids, intravenous immunoglobulin IVIg and plasmapheresis provide short-term benefits; IVIg is used for long-term maintenance.

Potential new therapeutic approaches involve targeting key factors in the immunopathogenesis of CIDP, including T cells, B cells and complement. Progress in clinical trial design is focused on clinically meaningful tools to define therapeutic responses, enrolling only patients with active disease and exploring biomarkers that predict response to therapies.

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Chronic inflammatory demyelinating polyradiculoneuropathy: from bench to bedside.

Published in Nature Reviews Neurology Marinos C. Skip to search form Skip to main content You are currently offline. Some features of the site may not work correctly. DOI: Chronic inflammatory demyelinating polyneuropathy CIDP is the most common chronic autoimmune neuropathy.

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Advances in the Diagnosis, Pathogenesis and Treatment of CIDP

Skip to search form Skip to main content You are currently offline. Some features of the site may not work correctly. DOI: Chronic inflammatory demyelinating polyneuropathy CIDP is the most common chronic autoimmune neuropathy.

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Advances in the diagnosis, pathogenesis and treatment of CIDP

Either your web browser doesn't support Javascript or it is currently turned off. In the latter case, please turn on Javascript support in your web browser and reload this page. Review Free to read. Multiple diagnostic criteria have been established, with the primary goal of identifying neurophysiologic hallmarks of acquired demyelination. Treatment modalities have expanded to include numerous immuno-modulatory therapies, although the best evidence continues to be for corticosteroids, plasma exchange, and intravenous immunoglobulins IVIg. This review describes the pathology, epidemiology, pathogenesis, diagnosis, and treatment of CIDP. The term CIDP was coined in by Peter Dyck and colleagues 1 , although similar remitting disorders were described by Eichhorst in and Henrikson in 2 and Austin in 3.

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