RT Journal Article
SR Electronic
T1 Effect of pulmonary exacerbations on long-term lung function decline in cystic fibrosis
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP erj01591-2011
DO 10.1183/09031936.00159111
A1 V. Waters
A1 S. Stanojevic
A1 E.G. Atenafu
A1 A. Lu
A1 Y. Yau
A1 E. Tullis
A1 F. Ratjen
YR 2012
UL http://erj.ersjournals.com/content/early/2011/12/01/09031936.00159111.abstract
AB It is unknown what proportion of long term lung function decline in cystic fibrosis (CF) is explained by pulmonary exacerbations. The aim of this study was to determine how exacerbations requiring hospitalization contribute to the course of CF lung disease .This was a retrospective cohort study. The primary outcome was the rate of decline of forced expiratory volume in 1 second percent predicted (FEV1% pred).Of 851 subjects, 415 (48.8%) subjects had ≥1 exacerbation. After adjustment for confounders, the annual rate of FEV1 decline in those without an exacerbation was 1.2%·yr−195% CI: 1.0;−1.5), compared with 2.5%·yr−195% CI: 2.1; 2.8) in those with an exacerbation. The proportion of overall FEV1 decline associated with ≥ 1 exacerbation was 52% (95% CI: 35.0; 68.9). For a given number of exacerbations, the annual rate of FEV1 decline was greatest in subjects with ≤ 6 months between exacerbations.Half of FEV1 decline seen in CF patients was associated with pulmonary exacerbations. Time between exacerbations, specifically ≤ 6 months between exacerbations, plays an important contribution to overall lung function decline. These findings support using time to next exacerbation as a clinical endpoint for CF trials.