Complement inhibitors in pediatric kidney diseases: new therapeutic opportunities.
Avacopan
C3 glomerulopathy
Children
Complement
Eculizumab
Kidney diseases
aHUS
Journal
Pediatric nephrology (Berlin, Germany)
ISSN: 1432-198X
Titre abrégé: Pediatr Nephrol
Pays: Germany
ID NLM: 8708728
Informations de publication
Date de publication:
May 2024
May 2024
Historique:
received:
02
05
2023
accepted:
01
08
2023
revised:
31
07
2023
medline:
18
3
2024
pubmed:
21
9
2023
entrez:
21
9
2023
Statut:
ppublish
Résumé
Historically, the complement system (classical, lectin, alternative, and terminal pathways) is known to play a crucial role in the etiopathogenesis of many kidney diseases. Direct or indirect activation in these settings is revealed by consumption of complement proteins at the serum level and kidney tissue deposition seen by immunofluorescence and electron microscopy. The advent of eculizumab has shown that complement inhibitors may improve the natural history of certain kidney diseases. Since then, the number of available therapeutic molecules and experimental studies on complement inhibition has increased exponentially. In our narrative review, we give a summary of the main complement inhibitors that have completed phase II and phase III studies or are currently used in adult and pediatric nephrology. The relevant full-text works, abstracts, and ongoing trials (clinicaltrials.gov site) are discussed. Data and key clinical features are reported for eculizumab, ravulizumab, crovalimab, avacopan, danicopan, iptacopan, pegcetacoplan, and narsoplimab. Many of these molecules have been shown to be effective in reducing proteinuria and stabilizing kidney function in different complement-mediated kidney diseases. Thanks to their efficacy and target specificity, these novel drugs may radically improve the outcome of complement-mediated kidney diseases, contributing to an improvement in our understanding of their underlying pathophysiology.
Identifiants
pubmed: 37733095
doi: 10.1007/s00467-023-06120-8
pii: 10.1007/s00467-023-06120-8
doi:
Substances chimiques
Complement Inactivating Agents
0
Complement C3
0
Types de publication
Review
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1387-1404Informations de copyright
© 2023. The Author(s), under exclusive licence to International Pediatric Nephrology Association.
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