Evolving Immunologic Perspectives in Chronic Inflammatory Demyelinating Polyneuropathy

Yusuf A Rajabally,1,2 Shahram Attarian,3,4 Emilien Delmont3,4 1Inflammatory Neuropathy Clinic, University Hospitals Birmingham, Birmingham, UK; 2Aston Medical School, Aston University, Birmingham, UK; 3Reference Centre for Neuromuscular Diseases and ALS, Centre Hospitalier Universitaire La Timone, M...

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Autores principales: Rajabally YA, Attarian S, Delmont E
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Publicado: Dove Medical Press 2020
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spelling oai:doaj.org-article:161c9bf6d2914d018c7772711b9f69e72021-12-02T15:36:49ZEvolving Immunologic Perspectives in Chronic Inflammatory Demyelinating Polyneuropathy1178-7031https://doaj.org/article/161c9bf6d2914d018c7772711b9f69e72020-09-01T00:00:00Zhttps://www.dovepress.com/evolving-immunologic-perspectives-in-chronic-inflammatory-demyelinatin-peer-reviewed-article-JIRhttps://doaj.org/toc/1178-7031Yusuf A Rajabally,1,2 Shahram Attarian,3,4 Emilien Delmont3,4 1Inflammatory Neuropathy Clinic, University Hospitals Birmingham, Birmingham, UK; 2Aston Medical School, Aston University, Birmingham, UK; 3Reference Centre for Neuromuscular Diseases and ALS, Centre Hospitalier Universitaire La Timone, Marseille 13385, France; 4Aix-Marseille University, Inserm, GMGF, Marseille, FranceCorrespondence: Yusuf A RajaballyAston Medical School, Aston University, Aston Triangle, Birmingham B4 7ET, UKEmail y.rajabally@aston.ac.ukAbstract: Chronic inflammatory demyelinating polyneuropathy (CIDP) is the commonest chronic idiopathic dysimmune neuropathy. Pathophysiologic processes involve both cellular and humoral immunity. There are various known forms of CIDP, likely caused by varying mechanisms. CIDP in its different forms is a treatable disorder in the majority of patients. The diagnosis of CIDP is clinical, supported routinely by electrophysiology. Cerebrospinal fluid analysis may be helpful. Routine immunology currently rarely adds to the diagnostic process but may contribute to the identification of an associated monoclonal gammopathy with or without hematologic malignancy and the consideration of alternative diagnoses, such as POEMS syndrome, anti-myelin associated glycoprotein (MAG) neuropathy or chronic ataxic neuropathy, with ophthalmoplegia, M-protein, cold aglutinins and disialosyl antibodies (CANOMAD). The search for antibodies specific to CIDP has been unsuccessful for many years. Recently, antibodies to paranodal proteins have been identified in a minority of patients with severe CIDP phenotypes, often unresponsive to first-line therapies. In conjunction with reports of high rates of antibody responses to neural structures in CIDP, this entertains the hope that more discoveries are to come. Although still arguably for only a small minority of patients, in view of current knowledge, such progress will enable earlier accurate diagnosis with direct management implications but only if the important, unfortunately and infrequently discussed issues of immunologic technique, test reliability and reproducibility are adequately tackled.Keywords: chronic inflammatory demyelinating polyneuropathy, dysimmune, immunologic, inflammatory, nodal, paranodalRajabally YAAttarian SDelmont EDove Medical Pressarticlechronic inflammatory demyelinating polyneuropathydysimmuneimmunologicinflammatorynodalparanodal.PathologyRB1-214Therapeutics. PharmacologyRM1-950ENJournal of Inflammation Research, Vol Volume 13, Pp 543-549 (2020)
institution DOAJ
collection DOAJ
language EN
topic chronic inflammatory demyelinating polyneuropathy
dysimmune
immunologic
inflammatory
nodal
paranodal.
Pathology
RB1-214
Therapeutics. Pharmacology
RM1-950
spellingShingle chronic inflammatory demyelinating polyneuropathy
dysimmune
immunologic
inflammatory
nodal
paranodal.
Pathology
RB1-214
Therapeutics. Pharmacology
RM1-950
Rajabally YA
Attarian S
Delmont E
Evolving Immunologic Perspectives in Chronic Inflammatory Demyelinating Polyneuropathy
description Yusuf A Rajabally,1,2 Shahram Attarian,3,4 Emilien Delmont3,4 1Inflammatory Neuropathy Clinic, University Hospitals Birmingham, Birmingham, UK; 2Aston Medical School, Aston University, Birmingham, UK; 3Reference Centre for Neuromuscular Diseases and ALS, Centre Hospitalier Universitaire La Timone, Marseille 13385, France; 4Aix-Marseille University, Inserm, GMGF, Marseille, FranceCorrespondence: Yusuf A RajaballyAston Medical School, Aston University, Aston Triangle, Birmingham B4 7ET, UKEmail y.rajabally@aston.ac.ukAbstract: Chronic inflammatory demyelinating polyneuropathy (CIDP) is the commonest chronic idiopathic dysimmune neuropathy. Pathophysiologic processes involve both cellular and humoral immunity. There are various known forms of CIDP, likely caused by varying mechanisms. CIDP in its different forms is a treatable disorder in the majority of patients. The diagnosis of CIDP is clinical, supported routinely by electrophysiology. Cerebrospinal fluid analysis may be helpful. Routine immunology currently rarely adds to the diagnostic process but may contribute to the identification of an associated monoclonal gammopathy with or without hematologic malignancy and the consideration of alternative diagnoses, such as POEMS syndrome, anti-myelin associated glycoprotein (MAG) neuropathy or chronic ataxic neuropathy, with ophthalmoplegia, M-protein, cold aglutinins and disialosyl antibodies (CANOMAD). The search for antibodies specific to CIDP has been unsuccessful for many years. Recently, antibodies to paranodal proteins have been identified in a minority of patients with severe CIDP phenotypes, often unresponsive to first-line therapies. In conjunction with reports of high rates of antibody responses to neural structures in CIDP, this entertains the hope that more discoveries are to come. Although still arguably for only a small minority of patients, in view of current knowledge, such progress will enable earlier accurate diagnosis with direct management implications but only if the important, unfortunately and infrequently discussed issues of immunologic technique, test reliability and reproducibility are adequately tackled.Keywords: chronic inflammatory demyelinating polyneuropathy, dysimmune, immunologic, inflammatory, nodal, paranodal
format article
author Rajabally YA
Attarian S
Delmont E
author_facet Rajabally YA
Attarian S
Delmont E
author_sort Rajabally YA
title Evolving Immunologic Perspectives in Chronic Inflammatory Demyelinating Polyneuropathy
title_short Evolving Immunologic Perspectives in Chronic Inflammatory Demyelinating Polyneuropathy
title_full Evolving Immunologic Perspectives in Chronic Inflammatory Demyelinating Polyneuropathy
title_fullStr Evolving Immunologic Perspectives in Chronic Inflammatory Demyelinating Polyneuropathy
title_full_unstemmed Evolving Immunologic Perspectives in Chronic Inflammatory Demyelinating Polyneuropathy
title_sort evolving immunologic perspectives in chronic inflammatory demyelinating polyneuropathy
publisher Dove Medical Press
publishDate 2020
url https://doaj.org/article/161c9bf6d2914d018c7772711b9f69e7
work_keys_str_mv AT rajaballyya evolvingimmunologicperspectivesinchronicinflammatorydemyelinatingpolyneuropathy
AT attarians evolvingimmunologicperspectivesinchronicinflammatorydemyelinatingpolyneuropathy
AT delmonte evolvingimmunologicperspectivesinchronicinflammatorydemyelinatingpolyneuropathy
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