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Differences in pulmonary exacerbation risks among CF subpopulations.

D R VanDevanter1, C O'Rourke2, M W Konstan3

  • 1Case Western Reserve University School of Medicine, Cleveland, OH, USA.

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
|November 30, 2025
PubMed
Summary
This summary is machine-generated.

Previous pulmonary exacerbations (PEx) in people with cystic fibrosis (pwCF) strongly predict future PEx risk, regardless of modulator therapy. Even with elexacaftor/tezacaftor/ivacaftor (ETI), higher prior PEx indicates greater future risk.

Keywords:
Clinical trial designPrior-year event frequencyPulmonary exacerbation risk

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Area of Science:

  • Pulmonary Medicine
  • Pharmacology
  • Epidemiology

Background:

  • Pulmonary exacerbations (PEx) are a key concern in cystic fibrosis (CF) management.
  • Assessing future PEx risk is crucial for treatment strategies.
  • The impact of newer CFTR modulator therapies, like elexacaftor/tezacaftor/ivacaftor (ETI), on PEx risk requires further investigation.

Purpose of the Study:

  • To evaluate the association between prior-year PEx frequency and future PEx risk in people with CF (pwCF).
  • To determine if this association persists among pwCF receiving ETI.
  • To inform the design of clinical trials for new CF therapies.

Main Methods:

  • Analysis of data from the CF Foundation Patient Registry (CFFPR) for pwCF aged 12+ years (2022-2023).
  • Stratification of 2022 PEx frequency (zero, one-two, three+).
  • Modeling of 2023 PEx hazards based on 2022 PEx frequency, disease stage, demographics, infection status, and modulator use (none vs. ETI).

Main Results:

  • Higher 2022 PEx frequency was strongly associated with increased mean 2023 PEx and shorter time to first 2023 PEx.
  • PwCF with three+ PEx in 2022 had a 7.11-fold higher hazard of PEx in 2023 compared to those with no PEx.
  • PwCF with one-two PEx in 2022 had a 2.89-fold higher hazard of PEx in 2023.

Conclusions:

  • Prior-year PEx frequency is a significant independent predictor of future PEx risk in pwCF.
  • ETI reduces PEx rates, but individuals with a history of higher PEx remain at elevated risk.
  • Future therapeutic interventions can be studied in ETI-treated pwCF with a history of frequent PEx.