Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy.
Обработка статьи · этапы, ошибки и управление
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Martín-Aguilar Lorena, Lleixà Cinta, Pascual-Goñi Elba, Caballero-Ávila Marta, Martínez-Martínez Laura, Díaz-Manera Jordi, Rojas-García Ricard, Cortés-Vicente Elena, Turon-Sans Janina, de Luna Noemi, Suárez-Calvet Xavier, Gallardo Eduard, Rajabally Yusuf, Scotton Sangeeta, Jacobs Bart C, Baars Adája, Cortese Andrea, Vegezzi Elisa, Höftberger Romana, Zimprich Fritz, Roesler Cornelia, Nobile-Orazio Eduardo, Liberatore Giuseppe, Hiew Fu Liong, Martínez-Piñeiro Alicia, Carvajal Alejandra, Piñar-Morales Raquel, Usón-Martín Mercedes, Albertí Olalla, López-Pérez Maria Ángeles, Márquez Fabian, Pardo-Fernández Julio, Muñoz-Delgado Laura, Cabrera-Serrano Macarena, Ortiz Nicolau, Bartolomé Manuel, Duman Özgür, Bril Vera, Segura-Chávez Darwin, Pitarokoili Kalliopi, Steen Claudia, Illa Isabel, Querol Luis
Аннотация
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: 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 < 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.
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.