Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy.
Adult
Aged
Autoantibodies
/ blood
Autoimmune Diseases of the Nervous System
/ blood
Cell Adhesion Molecules
/ immunology
Female
Humans
Immunologic Factors
/ pharmacology
Male
Middle Aged
Nerve Growth Factors
/ immunology
Ranvier's Nodes
/ immunology
Retrospective Studies
Rituximab
/ pharmacology
Young Adult
Journal
Neurology(R) neuroimmunology & neuroinflammation
ISSN: 2332-7812
Titre abrégé: Neurol Neuroimmunol Neuroinflamm
Pays: United States
ID NLM: 101636388
Informations de publication
Date de publication:
01 2022
01 2022
Historique:
received:
02
03
2021
accepted:
27
09
2021
entrez:
3
11
2021
pubmed:
4
11
2021
medline:
12
3
2022
Statut:
epublish
Résumé
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 ( 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.
Sections du résumé
BACKGROUND AND OBJECTIVES
To study the clinical and laboratory features of antineurofascin-155 (NF155)-positive autoimmune nodopathy (AN).
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 (
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.
Identifiants
pubmed: 34728497
pii: 9/1/e1098
doi: 10.1212/NXI.0000000000001098
pmc: PMC8564865
pii:
doi:
Substances chimiques
Autoantibodies
0
Cell Adhesion Molecules
0
Immunologic Factors
0
NFASC protein, human
0
Nerve Growth Factors
0
Rituximab
4F4X42SYQ6
Types de publication
Journal Article
Multicenter Study
Observational Study
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Commentaires et corrections
Type : ErratumIn
Informations de copyright
Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
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