Rationale: The prevalence of adults living with cystic fibrosis (CF) who have early-stage lung disease is increasing.
Objectives: Describe the prevalence and evaluate spirometric risk factors associated with the subgroup of patients with early-stage lung disease and FEV1 decline of ≥5% predicted/year.
Methods: Retrospective cohort study of patients ≥18 years with FEV1% predicted ≥80% included in the US CF Foundation Patient Registry from 2010-2013. Regression models were developed to estimate FEV1 rate of decline. Multivariable logistic analysis was used to assess if spirometric risk factors were associated with FEV1 decline.
Measurements and main results: 3,029 subjects were in the study cohort. Approximately 15% of the cohort had a substantial decline in lung function ≥5% predicted/year. In multivariable models adjusted for confounders, FEV1/FVC ratio <0.8 (Odds Ratio (OR) 1.63, 95% confidence interval (CI) 1.31 to 2.02) and history of FEV1% predicted variability (OR 2.35,95%CI 1.74 to 3.18) were associated with rapid lung function decline.
Conclusions: Even among adults with early-stage lung disease, approximately 15% are shown to progress and experience a large decline in lung function. This reinforces the concept that lung function in early-stage CF is not normal or mild. Rather, lung function decline may be delayed, but not avoided, in these individuals. Variability in FEV1% predicted and airway obstruction as measured by FEV1/FVC ratio may identify individuals at increased risk of decline. Adults with early-stage lung disease should be followed in clinic to monitor for onset of decline.
Keywords: Adult; Cohort studies; Cystic fibrosis; Respiratory function tests; Risk factors.
Copyright © 2019. Published by Elsevier B.V.
Is an FEV 1 of 80% Predicted a Normal Spirometry in Cystic Fibrosis Children and Adults?P König et al. Clin Respir J 12 (8), 2397-2403. PMID 29920961.Normal FEV1 , is not indicative of normal spirometry. FEV1 /FVC is the most sensitive measure of early or mild airway obstruction in children with c …
Persistent Methicillin-Resistant Staphylococcus Aureus and Rate of FEV1 Decline in Cystic FibrosisEC Dasenbrook et al. Am J Respir Crit Care Med 178 (8), 814-21. PMID 18669817.Persistent infection with MRSA in individuals with CF between the ages of 8 and 21 years is associated with a more rapid rate of decline in lung function.
Analysis of a Large Cohort of Cystic Fibrosis Patients With Severe Liver Disease Indicates Lung Function Decline Does Not Significantly Differ From That of the General Cystic Fibrosis PopulationD Polineni et al. PLoS One 13 (10), e0205257. PMID 30307979.Previous reports of lung function in cystic fibrosis (CF) patients with liver disease have shown worse, similar, or even better forced expiratory volume in 1 second (FEV1 …
Correctors (Specific Therapies for Class II CFTR Mutations) for Cystic FibrosisKW Southern et al. Cochrane Database Syst Rev 8 (8), CD010966. PMID 30070364. - ReviewThere is insufficient evidence that monotherapy with correctors has clinically important effects in people with CF who have two copies of the F508del mutation.Combination …
A Systematic Review of Studies Examining the Rate of Lung Function Decline in Patients With Cystic FibrosisSN Harun et al. Paediatr Respir Rev 20, 55-66. PMID 27259460. - ReviewA systematic review was performed (i) to describe the reported overall rate of progression of CF lung disease quantified as FEV1%predicted decline with age, (i …