%0 Journal Article
%A Martín-Aguilar, Lorena
%A Lleixà, Cinta
%A Pascual-Goñi, Elba
%A Caballero-Ávila, Marta
%A Martínez-Martínez, Laura
%A Díaz-Manera, Jordi
%A Rojas-García, Ricard
%A Cortés-Vicente, Elena
%A Turon-Sans, Janina
%A de Luna, Noemi
%A Suárez-Calvet, Xavier
%A Gallardo, Eduard
%A Rajabally, Yusuf
%A Scotton, Sangeeta
%A Jacobs, Bart C
%A Baars, Adája
%A Cortese, Andrea
%A Vegezzi, Elisa
%A Höftberger, Romana
%A Zimprich, Fritz
%A Roesler, Cornelia
%A Nobile-Orazio, Eduardo
%A Liberatore, Giuseppe
%A Hiew, Fu Liong
%A Martínez-Piñeiro, Alicia
%A Carvajal, Alejandra
%A Piñar-Morales, Raquel
%A Usón-Martín, Mercedes
%A Albertí, Olalla
%A López-Pérez, Maria Ángeles
%A Márquez, Fabian
%A Pardo-Fernández, Julio
%A Muñoz-Delgado, Laura
%A Cabrera-Serrano, Macarena
%A Ortiz, Nicolau
%A Bartolomé, Manuel
%A Duman, Özgür
%A Bril, Vera
%A Segura-Chávez, Darwin
%A Pitarokoili, Kalliopi
%A Steen, Claudia
%A Illa, Isabel
%A Querol, Luis
%T Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy.
%D 2021
%U http://hdl.handle.net/10668/20399
%X To study the clinical and laboratory features of antineurofascin-155 (NF155)-positive autoimmune nodopathy (AN). 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. Forty NF155+ patients with AN were included. Mean age at onset was 42.4 years. Patients presented with a progressive (75%), sensory motor (87.5%), and symmetric distal-predominant weakness in upper (97.2%) and lower extremities (94.5%), with tremor and ataxia (75%). Patients received a median of 3 (2-4) different treatments in 46 months of median follow-up. Response to IV immunoglobulin (86.8%) or steroids (72.2%) was poor in most patients, whereas 77.3% responded to rituximab. HLA-DRB1*15 was detected in 91.3% of patients. IgG4 anti-NF155 antibodies were predominant in all patients; anti-NF155 titers correlated with mRS within the same patient (r = 0.41, p = 0.004). Serum NfL (sNfL) levels were higher in anti-NF155+ AN than in healthy controls (36.47 vs 7.56 pg/mL, p  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. This study provides Class IV evidence that anti-NF155 antibodies associate with a specific phenotype and response to rituximab.
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