Neuromyelitis optica spectrum disorders: from pathophysiology to therapeutic strategies.
Aquaporin-4-antibody
Astrocyte
Complement
Neuroinflammation
Neuromyelitis optica spectrum disorders (NMOSD)
Ongoing trials
Treatment
Journal
Journal of neuroinflammation
ISSN: 1742-2094
Titre abrégé: J Neuroinflammation
Pays: England
ID NLM: 101222974
Informations de publication
Date de publication:
16 Sep 2021
16 Sep 2021
Historique:
received:
19
04
2021
accepted:
24
08
2021
entrez:
17
9
2021
pubmed:
18
9
2021
medline:
27
1
2022
Statut:
epublish
Résumé
Neuromyelitis optica (NMO) is a chronic inflammatory autoimmune disease of the central nervous system (CNS) characterized by acute optic neuritis (ON) and transverse myelitis (TM). NMO is caused by a pathogenic serum IgG antibody against the water channel aquoporin 4 (AQP4) in the majority of patients. AQP4-antibody (AQP4-ab) presence is highly specific, and differentiates NMO from multiple sclerosis. It binds to AQP4 channels on astrocytes, triggering activation of the classical complement cascade, causing granulocyte, eosinophil, and lymphocyte infiltration, culminating in injury first to astrocyte, then oligodendrocytes followed by demyelination and neuronal loss. NMO spectrum disorder (NMOSD) has recently been defined and stratified based on AQP4-ab serology status. Most NMOSD patients experience severe relapses leading to permanent neurologic disability, making suppression of relapse frequency and severity, the primary objective in disease management. The most common treatments used for relapses are steroids and plasma exchange.Currently, long-term NMOSD relapse prevention includes off-label use of immunosuppressants, particularly rituximab. In the last 2 years however, three pivotal clinical trials have expanded the spectrum of drugs available for NMOSD patients. Phase III studies have shown significant relapse reduction compared to placebo in AQP4-ab-positive patients treated with satralizumab, an interleukin-6 receptor (IL-6R) inhibitor, inebilizumab, an antibody against CD19
Identifiants
pubmed: 34530847
doi: 10.1186/s12974-021-02249-1
pii: 10.1186/s12974-021-02249-1
pmc: PMC8444436
doi:
Substances chimiques
AQP4 protein, human
0
Antibodies, Monoclonal, Humanized
0
Aquaporin 4
0
Autoantibodies
0
Immunosuppressive Agents
0
inebilizumab
74T7185BMM
eculizumab
A3ULP0F556
Azathioprine
MRK240IY2L
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
208Subventions
Organisme : Guthy-Jackson Charitable Foundation (US)
ID : Not applicable
Informations de copyright
© 2021. The Author(s).
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