Martín-Aguilar, LorenaLleixà, CintaPascual-Goñi, ElbaCaballero-Ávila, MartaMartínez-Martínez, LauraDíaz-Manera, JordiRojas-García, RicardCortés-Vicente, ElenaTuron-Sans, Janinade Luna, NoemiSuárez-Calvet, XavierGallardo, EduardRajabally, YusufScotton, SangeetaJacobs, Bart CBaars, AdájaCortese, AndreaVegezzi, ElisaHöftberger, RomanaZimprich, FritzRoesler, CorneliaNobile-Orazio, EduardoLiberatore, GiuseppeHiew, Fu LiongMartínez-Piñeiro, AliciaCarvajal, AlejandraPiñar-Morales, RaquelUsón-Martín, MercedesAlbertí, OlallaLópez-Pérez, Maria ÁngelesMárquez, FabianPardo-Fernández, JulioMuñoz-Delgado, LauraCabrera-Serrano, MacarenaOrtiz, NicolauBartolomé, ManuelDuman, ÖzgürBril, VeraSegura-Chávez, DarwinPitarokoili, KalliopiSteen, ClaudiaIlla, IsabelQuerol, Luis2023-05-032023-05-032021-11-02http://hdl.handle.net/10668/20399To 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.enAttribution-NonCommercial-NoDerivatives 4.0 Internationalhttp://creativecommons.org/licenses/by-nc-nd/4.0/AdultAgedAutoantibodiesAutoimmune Diseases of the Nervous SystemCell Adhesion MoleculesFemaleHumansImmunologic FactorsMaleMiddle AgedNerve Growth FactorsRanvier's NodesRetrospective StudiesRituximabYoung Adultresearch article34728497open access10.1212/NXI.00000000000010982332-7812PMC8564865https://nn.neurology.org/content/nnn/9/1/e1098.full.pdfhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8564865/pdf