Randomized trial reveals decreased inflammatory responses in classical monocytes after ETI therapy in PwCF, suggesting immunomodulatory effects.
Key Points
This research aims to explore the effects of Elexacaftor/Tezacaftor/Ivacaftor therapy on inflammatory responses in immune cells of cystic fibrosis patients.
Conducted single-cell RNA-sequencing on PBMC and lung explant tissues from healthy and cystic fibrosis donors.
Utilized bioinformatics for quality control, clustering, differential gene expression, and pathway analysis.
Compared inflammatory responses in classical monocytes before and after ETI therapy.
Significant suppression of LPS signaling, NF-κB, and MAPK/AP-1 pathways was observed in monocytes post-ETI therapy.
Down-regulation of CD14, TREM1 co-receptors and cytokines IL-1β and CXCL8 was noted, indicating reduced pro-inflammatory activity.
IL-1β production was normalized after therapy, highlighting potential biomarker opportunities.