Rituximab in treatment-resistant CIDP with antibodies against paranodal proteins.

Abstract

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.

• Authors: Luis Querol, Ricard Rojas-García, Jordi Diaz-Manera, Joseba Barcena, Julio Pardo, Angel Ortega-Moreno, Maria Jose Sedano, Laia Seró-Ballesteros, Alejandra Carvajal, Nicolau Ortiz, Eduard Gallardo, Isabel Illa
• Journal: Neurology(R) neuroimmunology & neuroinflammation (Neurol Neuroimmunol Neuroinflamm) Vol. 2 Issue 5 Pg. e149 (Oct 2015) ISSN: 2332-7812 [Print] United States
• PMID: 26401517 (Publication Type: Journal Article)