PT  - JOURNAL ARTICLE
AU  - Luis Querol
AU  - Ricard Rojas-García
AU  - Jordi Diaz-Manera
AU  - Joseba Barcena
AU  - Julio Pardo
AU  - Angel Ortega-Moreno
AU  - Maria Jose Sedano
AU  - Laia Seró-Ballesteros
AU  - Alejandra Carvajal
AU  - Nicolau Ortiz
AU  - Eduard Gallardo
AU  - Isabel Illa
TI  - Rituximab in treatment-resistant CIDP with antibodies against paranodal proteins
AID  - 10.1212/NXI.0000000000000149
DP  - 2015 Oct 01
TA  - Neurology - Neuroimmunology Neuroinflammation
PG  - e149
VI  - 2
IP  - 5
4099  - http://nn.neurology.org/content/2/5/e149.short
4100  - http://nn.neurology.org/content/2/5/e149.full
SO  - Neurol Neuroimmunol Neuroinflamm2015 Oct 01; 2
AB  - 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.CIDP=chronic inflammatory demyelinating polyneuropathy; CNTN1=contactin-1; IVIg=IV immunoglobulin; MuSK=muscle-specific tyrosine kinase; NF155=neurofascin-155; ONLS=Overall Neuropathy Limitations Scale; PEx=plasma exchange; PLA2R=M-type phospholipase A2 receptor; R-ODS=Rasch-built Overall Disability Scale