Observational studyClinical infectious diseases : an official publication of the Infectious Diseases Society of America2015
Pseudomonas aeruginosa in cystic fibrosis patients with G551D-CFTR treated with ivacaftor.
Observational study in Clinical infectious diseases : an official publication of the Infectious Diseases Society of America, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 132 papers, 1 of them a synthesis that pooled it.
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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.
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Who cites it
132 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Treatments for preventing recurrence of infection with Pseudomonas aeruginosa in people with cystic fibrosis.The Cochrane database of systematic reviews · 2019Pooled it
- Trial
- Clinical Effectiveness of Lumacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for F508del-CFTR. A Clinical Trial.Annals of the American Thoracic Society · 2021Trial
- Efficacy and Safety of the CFTR Potentiator Icenticaftor (QBW251) in COPD: Results from a Phase 2 Randomized Trial.International journal of chronic obstructive pulmonary disease · 2020Trial
- Adherence and barriers to hyperinsufflation in children with congenital muscular dystrophy.Pediatric pulmonology · 2017Trial
- How cystic fibrosis modulator therapy could transformMicrobiology (Reading, England) · 2026Review
- Article
- In Vitro Restoration of Colistin Susceptibility by Ivacaftor Synergy with Limited Reproducibility in a Murine Pneumonia Model.Antibiotics (Basel, Switzerland) · 2026Article
- PDE4 Inhibitor Apremilast Rebalances Inflammatory Responses to Pseudomonas aeruginosa Infection in CF Rats.bioRxiv : the preprint server for biology · 2026Article
- Human iPSC-derived macrophages for studying intrinsic and extrinsic factors in cystic fibrosis.EXO : beyond the cell · 2026Article
- The anti-inflammatory drug celecoxib is metabolized by Pseudomonas aeruginosa CYP107S1 in vitro and in vivo.Drug metabolism and disposition: the biological fate of chemicals · 2025Article
- Elevated glucose increasesInfection and immunity · 2025Article
- Article
- Article
- Effects of CFTR Modulators onInfectious disease reports · 2025Review
- Respiratory infections after elexacaftor/tezacaftor/ivacaftor treatment in people with cystic fibrosis: analysis of the European Cystic Fibrosis Society Patient Registry.ERJ open research · 2025Article
- Effects of Elevated Glucose on Bacterial Respiratory Infections in Cystic Fibrosis and Chronic Airway Diseases.International journal of molecular sciences · 2025Review
- Impact of Modulator Therapy on the Chronic Colonization of Lower Respiratory Tract Pathogens in Children: Data From Cystic Fibrosis Registry of Turkey.Pediatric pulmonology · 2025Article
- Article
- Recent developments inMicrobiology and molecular biology reviews : MMBR · 2025Review
72 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
8 authors.
Funding
Abstract
backgroundIvacaftor improves outcomes in cystic fibrosis (CF) patients with the G551D mutation; however, effects on respiratory microbiology are largely unknown. This study examines changes in CF respiratory pathogens with ivacaftor and correlates them with baseline characteristics and clinical response.
methodsThe G551D Observational Study enrolled a longitudinal observational cohort of US patients with CF aged 6 years and older with at least 1 copy of the G551D mutation. Results were linked with retrospective and prospective culture data in the US Cystic Fibrosis Foundation's National Patient Registry. Pseudomonas aeruginosa infection category in the year before and year after ivacaftor was compared and correlated with clinical findings.
resultsAmong 151 participants prescribed ivacaftor, 29% (26/89) who were culture positive for P. aeruginosa the year prior to ivacaftor use were culture negative the year following treatment; 88% (52/59) of those P. aeruginosa free remained uninfected. The odds of P. aeruginosa positivity in the year after ivacaftor compared with the year prior were reduced by 35% (odds ratio [OR], 0.65; P < .001). Ivacaftor was also associated with reduced odds of mucoid P. aeruginosa (OR, 0.77; P = .013) and Aspergillus (OR, 0.47; P = .039), but not Staphylococcus aureus or other common CF pathogens. Patients with intermittent culture positivity and higher forced expiratory volume in 1 second (FEV1) were most likely to turn culture negative. Reduction in P. aeruginosa was not associated with change in FEV1, body mass index, or hospitalizations.
conclusionsPseudomonas aeruginosa culture positivity was significantly reduced following ivacaftor treatment. Efficacious CFTR modulation may contribute to lower frequency of culture positivity for P. aeruginosa and other respiratory pathogens, particularly in patients with less established disease.
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