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Rituximab in treatment-resistant CIDP with antibodies against paranodal proteins

Lookup NU author(s): Professor Jordi Diaz ManeraORCiD



This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (CC BY-NC-ND).


Objective: To describe the response to rituximab in patients with treatment-resistant chronic inflammatory demyelinating polyneuropathy (CIDP) with antibodies against paranodal proteins and correlate the response with autoantibody titers. Methods: Patients with CIDP and IgG4 anti-contactin-1 (CNTN1) or anti-neurofascin-155 (NF155) antibodies who were resistant to IV immunoglobulin and corticosteroids were treated with rituximab and followed prospectively. Immunocytochemistry was used to detect anti-CNTN1 and anti-NF155 antibodies and ELISA with human recombinant CNTN1 and NF155 proteins was used to determine antibody titers. Results: Two patients had a marked improvement; another patient improved slightly after 10 years of stable, severe disease; and the fourth patient had an ischemic stroke unrelated to treatment and was lost to follow-up. Autoantibodies decreased in all patients after rituximab treatment. Conclusions: Rituximab treatment is an option for patients with CIDP with IgG4 anti-CNTN1/NF155 antibodies who are resistant to conventional therapies. Classification of evidence: This study provides Class IV evidence that rituximab is effective for patients with treatment-resistant CIDP with IgG4 anti-CNTN1 or anti-NF155 antibodies.

Publication metadata

Author(s): Querol L, Rojas-Garcia R, Diaz-Manera J, Barcena J, Pardo J, Ortega-Moreno A, Sedano MJ, Sero-Ballesteros L, Carvajal A, Ortiz N, Gallardo E, Illa I

Publication type: Article

Publication status: Published

Journal: Neurology: Neuroimmunology and NeuroInflammation

Year: 2015

Volume: 2

Issue: 5

Print publication date: 01/10/2015

Online publication date: 03/09/2015

Acceptance date: 15/07/2015

Date deposited: 25/02/2020

ISSN (electronic): 2332-7812

Publisher: Lippincott Williams and Wilkins


DOI: 10.1212/NXI.0000000000000149


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