Observational studyThe European respiratory journal2025
Reduction of systemic inflammation by elexacaftor/tezacaftor/ivacaftor correlates with lung function improvement in cystic fibrosis.
Observational study in The European respiratory journal, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed.
- Anti-inflammatory and phosphorylation effects of CFTR modulator triple therapy in cystic fibrosis.iScience · 2026Article
- Understanding Nasal Polyposis: The Roles of Ion Channels, Inflammation, Ionocytes, and Prostaglandin E2-The IJournal of clinical medicine · 2026Article
- Changes of inflammatory markers in nasal lavage samples after 2 years of elexacaftor/tezacaftor/ivacaftor therapy in paediatric and adult cystic fibrosis patients.ERJ open research · 2026Article
- Pediatric cystic fibrosis is associated with type 3 immune response.Molecular biology reports · 2026Article
- Antiviral defenses are diminished at birth in cystic fibrosis pig airways.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2026Article
- Evolving Cystic Fibrosis Therapy: The Good, the Sad, and the Hopeful.Children (Basel, Switzerland) · 2026Review
- The cystic fibrosis airway cytokine interleukin-1β enhances cystic fibrosis transmembrane conductance regulator modulator efficacy.ERJ open research · 2026Article
- Type 3 innate lymphoid cells dominate the ILC compartment in endstage lung disease.Frontiers in immunology · 2026Article
- Evolving Cystic Fibrosis Care: Lung Immunology and Emerging Health Challenges in the Era of CFTR Modulators.Biomolecules · 2025Review
- Iron Supplementation in Anemic Patients with Cystic Fibrosis: Effects on Hematologic, Clinical, and Pulmonary Outcomes.Tanaffos · 2025Article
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Authors and funding
36 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThe triple cystic fibrosis transmembrane conductance regulator modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) rapidly improves airway and systemic inflammation in people with cystic fibrosis. However, longitudinal effects on systemic inflammation and their relationship to lung function remain unknown.
methodsIn this prospective, observational, multicentre study, we analysed peripheral blood neutrophil counts, C-reactive protein (CRP) and six pro-inflammatory serum cytokines in a cohort of 198 people with cystic fibrosis aged ≥6 years at baseline and follow-up visits 3, 12 and 24 months after initiation of ETI, compared to 74 age-matched healthy control participants.
resultsNeutrophil counts and CRP, granulocyte colony-stimulating factor, interleukin (IL)-1β, IL-6 and IL-8 were reduced to 71%, 40%, 41%, 63%, 46% and 81% of median baseline values, respectively, after 3 months of therapy (all p<0.05), whereas monocyte chemotactic protein-1 reached 82% of baseline levels at 12 months only (p<0.05). Change from baseline to 3 months correlated with improvements in percent predicted forced expiratory volume for all systemic inflammation parameters except IL-8 (Spearman's r -0.17 to -0.42, p<0.05). All cytokines reached healthy control levels at or before 24 months. Decreased inflammation levels were sustained until 24 months for all parameters (p<0.05) except IL-6.
conclusionsOur results demonstrate that ETI exerts rapid and sustained effects on systemic inflammation associated with lung function improvements in children, adolescents and adults with cystic fibrosis in a real-world, post-approval setting. However, our data also show that individual markers of systemic inflammation remain at levels above those of healthy controls, particularly in certain subgroups, suggesting persistence or resurgence of residual systemic inflammation.
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