' '
Deutsch | English    

Forschungsdatenbank PMU-SQQUID

Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy.
Martin-Aguilar, L; Lleixa, C; Pascual-Goni, E; Caballero-Avila, M; Martinez-Martinez, L; Diaz-Manera, J; Rojas-Garcia, R; Cortes-Vicente, E; Turon-Sans, J; de Luna, N; Suarez-Calvet, X; Gallardo, E; Rajabally, Y; Scotton, S; Jacobs, BC; Baars, A; Cortese, A; Vegezzi, E; Hoftberger, R; Zimprich, F; Roesler, C; Nobile-Orazio, E; Liberatore, G; Hiew, FL; Martinez-Pineiro, A; Carvajal, A; Pinar-Morales, R; Uson-Martin, M; Alberti, O; Lopez-Perez, MA; Marquez, F; Pardo-Fernandez, J; Munoz-Delgado, L; Cabrera-Serrano, M; Ortiz, N; Bartolome, M; Duman, O; Bril, V; Segura-Chavez, D; Pitarokoili, K; Steen, C; Illa, I; Querol, L
Originalarbeiten (Zeitschrift)


Rösler Cornelia


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 < 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.
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.

Useful keywords (using NLM MeSH Indexing)




Autoimmune Diseases of the Nervous System*/blood

Autoimmune Diseases of the Nervous System*/drug therapy

Autoimmune Diseases of the Nervous System*/immunology

Autoimmune Diseases of the Nervous System*/physiopathology

Cell Adhesion Molecules/immunology*



Immunologic Factors/pharmacology*


Middle Aged

Nerve Growth Factors/immunology*

Ranvier"s Nodes/immunology*

Retrospective Studies


Young Adult