Autoantibodies Against the Node of Ranvier in Seropositive Chronic Inflammatory Demyelinating Polyneuropathy: Diagnostic, Pathogenic, and Therapeutic Relevance

Bibliographic Details
Title: Autoantibodies Against the Node of Ranvier in Seropositive Chronic Inflammatory Demyelinating Polyneuropathy: Diagnostic, Pathogenic, and Therapeutic Relevance
Authors: Atay Vural, Kathrin Doppler, Edgar Meinl
Source: Frontiers in Immunology, Vol 9 (2018)
Publisher Information: Frontiers Media S.A., 2018.
Publication Year: 2018
Collection: LCC:Immunologic diseases. Allergy
Subject Terms: autoantibody, seropositive, chronic inflammatory demyelinating polyneuropathy, node of Ranvier, paranode, neurofascin, Immunologic diseases. Allergy, RC581-607
More Details: Discovery of disease-associated autoantibodies has transformed the clinical management of a variety of neurological disorders. Detection of autoantibodies aids diagnosis and allows patient stratification resulting in treatment optimization. In the last years, a set of autoantibodies against proteins located at the node of Ranvier has been identified in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). These antibodies target neurofascin, contactin1, or contactin-associated protein 1, and we propose to name CIDP patients with these antibodies collectively as seropositive. They have unique clinical characteristics that differ from seronegative CIDP. Moreover, there is compelling evidence that autoantibodies are relevant for the pathogenesis. In this article, we review the current knowledge on the characteristics of autoantibodies against the node of Ranvier proteins and their clinical relevance in CIDP. We start with a description of the structure of the node of Ranvier followed by a summary of assays used to identify seropositive patients; and then, we describe clinical features and characteristics linked to seropositivity. We review knowledge on the role of these autoantibodies for the pathogenesis with relevance for the emerging concept of nodopathy/paranodopathy and summarize the treatment implications.
Document Type: article
File Description: electronic resource
Language: English
ISSN: 1664-3224
Relation: http://journal.frontiersin.org/article/10.3389/fimmu.2018.01029/full; https://doaj.org/toc/1664-3224
DOI: 10.3389/fimmu.2018.01029
Access URL: https://doaj.org/article/fdcbd509a6844bdc8eb28e17fca03bf4
Accession Number: edsdoj.fdcbd509a6844bdc8eb28e17fca03bf4
Database: Directory of Open Access Journals
More Details
ISSN:16643224
DOI:10.3389/fimmu.2018.01029
Published in:Frontiers in Immunology
Language:English