Rituximab in treatment-resistant CIDP with antibodies against paranodal proteins

L Querol, R Rojas-García, J Diaz-Manera… - Neuroimmunology & …, 2015 - AAN Enterprises
L Querol, R Rojas-García, J Diaz-Manera, J Barcena, J Pardo, A Ortega-Moreno, MJ Sedano…
Neuroimmunology & Neuroinflammation, 2015AAN Enterprises
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 …
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.
American Academy of Neurology