A 3-year prognostic score for adults with cystic fibrosis

J Cyst Fibros. 2017 Nov;16(6):702-708. doi: 10.1016/j.jcf.2017.03.004. Epub 2017 Mar 18.


Background: Therapeutic progress in patients with cystic fibrosis (CF) has resulted in improved prognosis over the past decades. We aim to reevaluate prognostic factors of CF and provide a prognostic score to predict the risk of death or lung transplantation (LT) within a 3-year period in adult patients.

Methods: We developed a logistic model using data from the French CF Registry and combined the coefficients into a prognostic score. The discriminative abilities of the model and the prognostic score were assessed by c-statistic. The prognostic score was validated using a 10-fold cross-validation.

Results: The risk of death or LT within 3years was related to eight characteristics. The development and the validation provided excellent results for the prognostic score; the c-statistic was 0.91 and 0.90 respectively.

Conclusion: The score developed to predict 3-year death or LT in adults with CF might be useful for clinicians to identify patients requiring specialized evaluation for LT.

Keywords: Cystic fibrosis; Logistic model; Prognostic factors; Prognostic score; Registry data.

MeSH terms

  • Adult
  • Cystic Fibrosis* / diagnosis
  • Cystic Fibrosis* / mortality
  • Cystic Fibrosis* / therapy
  • Female
  • Forced Expiratory Volume
  • France / epidemiology
  • Humans
  • Lung Transplantation* / methods
  • Lung Transplantation* / statistics & numerical data
  • Male
  • Patient Selection
  • Prognosis
  • Registries / statistics & numerical data
  • Research Design
  • Risk Assessment / methods
  • Risk Assessment / statistics & numerical data
  • Survival Analysis
  • Time-to-Treatment