RT Journal Article
T1 Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy.
A1 Martín-Aguilar, Lorena
A1 Lleixà, Cinta
A1 Pascual-Goñi, Elba
A1 Caballero-Ávila, Marta
A1 Martínez-Martínez, Laura
A1 Díaz-Manera, Jordi
A1 Rojas-García, Ricard
A1 Cortés-Vicente, Elena
A1 Turon-Sans, Janina
A1 de Luna, Noemi
A1 Suárez-Calvet, Xavier
A1 Gallardo, Eduard
A1 Rajabally, Yusuf
A1 Scotton, Sangeeta
A1 Jacobs, Bart C
A1 Baars, Adája
A1 Cortese, Andrea
A1 Vegezzi, Elisa
A1 Höftberger, Romana
A1 Zimprich, Fritz
A1 Roesler, Cornelia
A1 Nobile-Orazio, Eduardo
A1 Liberatore, Giuseppe
A1 Hiew, Fu Liong
A1 Martínez-Piñeiro, Alicia
A1 Carvajal, Alejandra
A1 Piñar-Morales, Raquel
A1 Usón-Martín, Mercedes
A1 Albertí, Olalla
A1 López-Pérez, Maria Ángeles
A1 Márquez, Fabian
A1 Pardo-Fernández, Julio
A1 Muñoz-Delgado, Laura
A1 Cabrera-Serrano, Macarena
A1 Ortiz, Nicolau
A1 Bartolomé, Manuel
A1 Duman, Özgür
A1 Bril, Vera
A1 Segura-Chávez, Darwin
A1 Pitarokoili, Kalliopi
A1 Steen, Claudia
A1 Illa, Isabel
A1 Querol, Luis
AB 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.
YR 2021
FD 2021-11-02
LK http://hdl.handle.net/10668/20399
UL http://hdl.handle.net/10668/20399
LA en
DS RISalud
RD Jul 30, 2025