Observational studyAnnals of the American Thoracic Society2026
Long-term reductions in inflammation in people with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor.
Observational study in Annals of the American Thoracic Society, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT04038047 (A Prospective Study to Evaluate Biological and Clinical Effects of Significantly Corrected CFTR Function), which is not on this map. Cited by 12 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
A Prospective Study to Evaluate Biological and Clinical Effects of Significantly Corrected CFTR Function (The PROMISE Study)
Who cites it
12 citing papers in PubMed.
- The impact of modulator therapy on theProceedings of the National Academy of Sciences of the United States of America · 2026Article
- The dipeptidyl peptidase 1 inhibition landscape in bronchiectasis and its future direction.European respiratory review : an official journal of the European Respiratory Society · 2026Review
- Anti-inflammatory and phosphorylation effects of CFTR modulator triple therapy in cystic fibrosis.iScience · 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
- Patient-Reported Health-Related Quality of Life in Romanian Patients with Cystic Fibrosis in the Era of Highly Effective CFTR Modulator Therapy: A National Cross-Sectional Mixed-Methods Survey.Journal of clinical medicine · 2026Article
- Evolving Cystic Fibrosis Therapy: The Good, the Sad, and the Hopeful.Children (Basel, Switzerland) · 2026Review
- Function and Structure Relationships With Inflammation Differ in Two Chronic Suppurative Lung Diseases.Pediatric pulmonology · 2026Article
- Functional CFTR may be required for Prevotella melaninogenica regulation of epithelial cell defense against Staphylococcus aureus.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2026Article
- PDE4 Inhibitor Apremilast Rebalances Inflammatory Responses to Pseudomonas aeruginosa Infection in CF Rats.bioRxiv : the preprint server for biology · 2026Article
- Sputum Biomarkers of Inflammation to Track Acute Respiratory Events in School-Age Children with Cystic Fibrosis.International journal of molecular sciences · 2026Observational
- Modulation of immune responses by elexacaftor/tezacaftor/ivacaftor therapy in cystic fibrosis: data from a compassionate use program.Respiratory research · 2026Article
- Modulation of host inflammatory pathways byFrontiers in immunology · 2026Article
Corrections and comments
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Authors and funding
16 authors.
Funding
Abstract
rationaleInflammation is a hallmark of cystic fibrosis (CF) and associated with bronchiectasis and lung disease progression. The effects of elexacaftor/tezacaftor/ivacaftor (ETI), a CF transmembrane conductance regulator modulator therapy, on inflammation remain incompletely understood.
objectivesInvestigate 2-year changes in airway and systemic inflammation in adolescents and adults with CF clinically prescribed ETI and the relationships between inflammatory changes and clinical outcomes.
methodsPROMISE is a prospective, multicenter, observational study in people with CF aged ≥12 years. Assessments of sputum and blood inflammatory markers occurred before and through 24-30 months of ETI therapy in participants who enrolled in the PROMISE-Inflammation substudy. Changes in inflammation were tested with mixed-effects models. Relationships between inflammatory markers and clinical outcomes were examined using Spearman correlations.
resultsThe study cohort comprised 223 participants. ETI was associated with sustained reductions in sputum neutrophil elastase activity, calprotectin, IL-1β, and IL-8, increases in sputum IL-6 through 24/30 months of therapy, and reductions in circulating high-sensitivity CRP (hsCRP) through 12/18 months of therapy. Sputum NE activity reductions correlated with percent predicted forced expiratory volume in 1 second (ppFEV1) and respiratory symptom score improvements at 24/30 months post-ETI. Sputum IL-6 increases correlated with ppFEV1 improvements. Serum hsCRP reductions were associated with ppFEV1 and respiratory symptoms improvements at 12/18 months post-ETI, and circulating calprotectin reductions were associated with respiratory symptom improvements.
conclusionsAirway and systemic inflammation decreases through 2.5 years of ETI therapy in adolescents and adults with CF. Reductions in inflammation correlate with clinical improvements. These changes in inflammation represent a disease-modifying benefit of this transformative therapy. CLINICAL TRIALS REGISTRATION: NCT04038047.
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