Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy
L. Aguilar, Cinta Lleixà, Elba Pascual‐Goñi, Marta Caballero‐Ávila, Laura Martínez‐Martínez, Jordi Díaz‐Manera, Ricard Rojas‐García, Elena Cortés‐Vicente, Janina Turón‐Sans, Noemí de Luna, Xavier Suárez‐Calvet, Eduard Gallardo, Yusuf A. Rajabally, Sangeeta Scotton, Bart C. Jacobs, Adája E. Baars, Andrea Cortese, Elisa Vegezzi, Romana Höftberger, Fritz Zimprich, Cornelia Roesler, Eduardo Nobile‐Orazio, Giuseppe Liberatore, Fu Liong Hiew, Alicia Martínez‐Piñeiro, Alejandra Carvajal, Raquel Piñar Morales, Mercedes Usón‐Martín, Olalla Albertí, Maria Angeles López‐Pérez, Fabián Márquez, Julio Pardo-Fernández, Laura Muñoz‐Delgado, Macarena Cabrera‐Serrano, Nicolau Ortiz, Manuel Bartolomé, Özgür Duman, Vera Bril, Darwin Segura-Chávez, Kalliopi Pitarokoili, Claudia Steen, Isabel Illa, Luís Querol
Abstract
BACKGROUND AND OBJECTIVES: To study the clinical and laboratory features of antineurofascin-155 (NF155)-positive autoimmune nodopathy (AN). METHODS: Patients with anti-NF155 antibodies detected on routine immunologic testing were included. Clinical characteristics, treatment response, and functional scales (modified Rankin Scale [mRS] and Inflammatory Rasch-built Overall Disability Scale [I-RODS]) were retrospectively collected at baseline and at the follow-up. Autoantibody and neurofilament light (NfL) chain levels were analyzed at baseline and at the follow-up. RESULTS: = 0.01). Anti-NF155 titers and sNfL levels decreased in all rituximab-treated patients. DISCUSSION: Anti-NF155 AN presents a distinct clinical profile and good response to rituximab. Autoantibody titers and sNfL are useful to monitor disease status in these patients. The use of untagged-NF155 plasmids minimizes the detection of false anti-NF155+ cases. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that anti-NF155 antibodies associate with a specific phenotype and response to rituximab.