Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy

Author:

Martín-Aguilar LorenaORCID,Lleixà Cinta,Pascual-Goñi Elba,Caballero-Ávila MartaORCID,Martínez-Martínez Laura,Díaz-Manera Jordi,Rojas-García RicardORCID,Cortés-Vicente Elena,Turon-Sans Janina,de Luna Noemi,Suárez-Calvet XavierORCID,Gallardo Eduard,Rajabally Yusuf,Scotton Sangeeta,Jacobs Bart C.ORCID,Baars AdájaORCID,Cortese Andrea,Vegezzi ElisaORCID,Höftberger Romana,Zimprich FritzORCID,Roesler Cornelia,Nobile-Orazio Eduardo,Liberatore GiuseppeORCID,Hiew Fu Liong,Martínez-Piñeiro Alicia,Carvajal Alejandra,Piñar-Morales RaquelORCID,Usón-Martín Mercedes,Albertí Olalla,López-Pérez Maria Ángeles,Márquez Fabian,Pardo-Fernández Julio,Muñoz-Delgado LauraORCID,Cabrera-Serrano Macarena,Ortiz Nicolau,Bartolomé Manuel,Duman Özgür,Bril Vera,Segura-Chávez Darwin,Pitarokoili Kalliopi,Steen Claudia,Illa Isabel,Querol LuisORCID

Abstract

Background and ObjectivesTo study the clinical and laboratory features of antineurofascin-155 (NF155)–positive autoimmune nodopathy (AN).MethodsPatients 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.ResultsForty 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 < 0.001) and correlated with anti-NF155 titers (r = 0.43, p = 0.001), with I-RODS at baseline (r = −0.88, p < 0.001) and with maximum I-RODS achieved (r = −0.58, p = 0.01). Anti-NF155 titers and sNfL levels decreased in all rituximab-treated patients.DiscussionAnti-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 EvidenceThis study provides Class IV evidence that anti-NF155 antibodies associate with a specific phenotype and response to rituximab.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Neurology (clinical),Neurology

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