Neuromuscular 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.
Neurol Neuroimmunol Neuroinflamm. 2015 Sep 3;2(5):e149. doi: 10.1212/NXI.0000000000000149. eCollection 2015 Oct.
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.
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.
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.
Rituximab treatment is an option for patients with CIDP with IgG4 anti-CNTN1/NF155 antibodies who are resistant to conventional therapies.
This study provides Class IV evidence that rituximab is effective for patients with treatment-resistant CIDP with IgG4 anti-CNTN1 or anti-NF155 antibodies.
描述针对伴有抗节细胞蛋白抗体的治疗抵抗性慢性炎症性脱髓鞘性多发性神经病（CIDP）患者使用利妥昔单抗的反应，并将其与自身抗体滴度相关联。
对 IgG4 抗接触蛋白-1（CNTN1）或抗神经束蛋白-155（NF155）抗体、对静脉注射免疫球蛋白和皮质类固醇治疗抵抗的 CIDP 患者，使用利妥昔单抗进行治疗并前瞻性随访。使用免疫细胞化学检测抗 CNTN1 和抗 NF155 抗体，用人重组 CNTN1 和 NF155 蛋白的 ELISA 法测定抗体滴度。
2 例患者有明显改善；另 1 例在 10 年稳定、严重疾病后略有改善；第 4 例患者因与治疗无关的缺血性卒中而失访。利妥昔单抗治疗后，所有患者的自身抗体滴度均降低。
对于对常规治疗抵抗的 IgG4 抗 CNTN1/NF155 抗体的 CIDP 患者，利妥昔单抗治疗是一种选择。
本研究提供了 IV 级证据，表明利妥昔单抗对 IgG4 抗 CNTN1 或抗 NF155 抗体、治疗抵抗性 CIDP 患者有效。
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