Rituximab in treatment-resistant CIDP with antibodies against paranodal proteins
Luis Querol1, Ricard Rojas-García1, Jordi Diaz-Manera1
1Neuromuscular Diseases Unit (L.Q., R.R.-G., J.D.-M., E.G., I.I.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro para la Investigación Biomédica en Red en Enfermedades Raras (L.Q., R.R.-G., J.D.-M., E.G., I.I.), CIBERER, Madrid, Spain; Neurology Department (J.B.), Hospital Universitario de Cruces, Universidad del País Vasco, Spain; Department of Neurology (J.P.), Hospital Clínico de Santiago, Santiago de Compostela, Spain; Department of Neurology (A.O.-M., A.C.), Hospital Virgen de las Nieves, Granada, Spain; Department of Neurology (M.J.S.), University Hospital "Marqués de Valdecilla" (IFIMAV) and University of Cantabria, Santander, Spain; Department of Neurology (L.S.-B.), Hospital Univeristari Vall d'Hebrón, Universitat Autònoma de Barcelona, Barcelona, Spain; and Department of Neurology (UHN) (N.O.), Hospital Universitari Sant Joan, Universitat Rovira i Virgili, Reus, Spain.
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.
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