%0 Journal Article %A Querol, Luis %A Rojas-García, Ricard %A Diaz-Manera, Jordi %A Barcena, Joseba %A Pardo, Julio %A Ortega-Moreno, Angel %A Sedano, Maria Jose %A Seró-Ballesteros, Laia %A Carvajal, Alejandra %A Ortiz, Nicolau %A Gallardo, Eduard %A Illa, Isabel %T Rituximab in treatment-resistant CIDP with antibodies against paranodal proteins. %D 2015 %U http://hdl.handle.net/10668/2063 %X OBJECTIVETo 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.METHODSPatients 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.RESULTSTwo 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.CONCLUSIONSRituximab treatment is an option for patients with CIDP with IgG4 anti-CNTN1/NF155 antibodies who are resistant to conventional therapies.CLASSIFICATION OF EVIDENCEThis study provides Class IV evidence that rituximab is effective for patients with treatment-resistant CIDP with IgG4 anti-CNTN1 or anti-NF155 antibodies. %K Corticoesteroides %K Anticuerpos %K Anticuerpos monoclonales de origen murino %K Autoanticuerpos %K Contactina 1 %K Ensayo de inmunoadsorción enzimática %K Humanos %K Inmunoglobulina G %K Inmunoglobulinas intravenosas %K Inmunohistoquímica %K Perdida de seguimiento %K Polirradiculoneuropatía crónica inflamatoria desmielinizante %K Accidente cerebrovascular %~