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Nat Rev Neurol. 2017 Sep;13(9):533-547. doi: 10.1038/nrneurol.2017.84. Epub 2017 Jul 14.

Autoantibodies in chronic inflammatory neuropathies: diagnostic and therapeutic implications.

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Neuromuscular Diseases Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Mas Casanovas 90, 08041 Barcelona, Spain.
Centro para la Investigación Biomédica en Red en Enfermedades Raras, CIBERER (Centre for Biomedical Network Research on Rare Diseases), Avenida Monforte de Lemos 6, 28029 Madrid, Spain.
Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), CRN2M-UMR7286, Faculté de Médecine - Secteur Nord CS80011, 51 Boulevard Pierre Dramard 13344 MARSEILLE Cedex 15, France.


The chronic inflammatory neuropathies (CINs) are rare, very disabling autoimmune disorders that generally respond well to immune therapies such as intravenous immunoglobulin (IVIg). The most common forms of CIN are chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy, and polyneuropathy associated with monoclonal gammopathy of unknown significance. The field of CIN has undergone a major advance with the identification of IgG4 autoantibodies directed against paranodal proteins in patients with CIDP. Although these autoantibodies are only found in a small subset of patients with CIDP, they can be used to guide therapeutic decision-making, as these patients have a poor response to IVIg. These observations provide proof of concept that identifying the target antigens in tissue-specific antibody-mediated autoimmune diseases is important, not only to understand their underlying pathogenic mechanisms, but also to correctly diagnose and treat affected patients. This state-of-the-art Review focuses on the role of autoantibodies against nodes of Ranvier in CIDP, a clinically relevant emerging field of research. The role of autoantibodies in other immune-mediated neuropathies, including other forms of CIN, primary autoimmune neuropathies, neoplasms, and systemic diseases that resemble CIN, are also discussed.


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