Predicting disease progression in cystic fibrosis. (2nd November 2018)

• Record Type: Journal Article
• Authors: Breuer, Oded; Caudri, Daan; Stick, Stephen; Turkovic, Lidija
• Is Part Of: Expert review of respiratory medicine. Volume 12:Number 11(2018)
• Page Start: 905
• Page End: 917
• Publication Date: 2018-11-02
• Subjects: FEV1% predicted -- Lung clearance index (LCI) -- Chest CT scores; Respiratory organs -- Diseases -- Periodicals; Respiratory organs -- Diseases -- Treatment -- Periodicals; 616.2005
• DOI: 10.1080/17476348.2018.1519400
• Languages: English
• ISSNs: 1747-6348

Abstract

ABSTRACT: Introduction : Progressive lung disease is the major cause of morbidity and mortality in patients with cystic fibrosis (CF). Methods of correctly predicting the future progression of lung disease in patients with CF are essential for directing aggressive treatment to prevent loss of lung function and end stage respiratory failure. Areas covered : This review addresses predictors of respiratory disease progression in patients with CF. We searched Web of Science and Medline, with no restriction on publication date, with the search terms 'cystic fibrosis' and 'disease progression', 'lung function decline', 'prognosis', 'prediction/predictive', 'prediction/prognostic scores', 'risk factors', 'outcome measures/endpoints/disease surrogate', 'longitudinal/long term', 'statistical model', and 'survival'. Expert commentary : Forced expiratory volume in 1 sec (FEV1 ) and rate of FEV1 decline, remain the most significant predictors of mortality in patients with CF while CT scores and airway secretion biomarkers are the main predictors of early CF lung disease. Comprehensive scores incorporating clinical, lung function, imaging and laboratory data will become essential in the future for predicting disease progression and for use in clinical trials. Early interventions may delay the progression of structural lung disease.