Abstract
Background: Forced expiratory volume 1 second (FEV1) has traditionally been used as a readily available marker of health in adult cystic fibrosis (CF). However, due to the obstructive nature of this disease, it is possible that lung hyperinflation could be more closely related to disease severity than is FEV1. The purpose of this study was to determine if hyperinflation is more closely associated with quality of life, functional status, and pulmonary exacerbations than FEV1 in patients with CF. Methods: Sixty-eight adult patients with CF were evaluated in this retrospective study. We used IC and functional residual capacity (FRC) and their ratios to total lung capacity (TLC) as measures of lung hyperinflation. We used bivariate correlations and backwards regression analysis to assess possible associations between FEV1, lung hyperinflation, and measures of disease severity including questionnaire based quality of life, pulmonary exacerbation frequency, and mortality. The respiratory component of the Cystic Fibrosis Questionnaire–Revised (CRQ-R-Respiratory) was used as a measure of quality of life. Results: Both FEV1 and IC were negatively correlated with pulmonary exacerbations over a 3 year period (p = 0.004, r2 = 0.127; p Conclusions: FEV1 and lung hyperinflation-as measured by IC and FRC/TLC-are both associated with pulmonary exacerbation frequency. This suggests that chronic dynamic hyperinflation contributes significantly to disease severity in adult cystic fibrosis.
Highlights
Cystic fibrosis (CF) is a progressive, multisystem disorder characterized by excessive production of thickened exocrine secretions
Studies in COPD show that tidal expiratory flow limitation is more closely related to dyspnea than is Forced expiratory volume 1 second (FEV1) [8]
The purpose of this study was to determine if hyperinflation is more closely associated with functional status and pulmonary exacerbations than is FEV1 in adult patients with cystic fibrosis (CF), using inspiratory capacity (IC), functional residual capacity (FRC), residual volume (RV), and their ratios to total lung capacity (TLC) as indices of hyperinflation
Summary
Cystic fibrosis (CF) is a progressive, multisystem disorder characterized by excessive production of thickened exocrine secretions. Others have shown that the ratio of IC to total lung capacity (IC/TLC) is an independent predictor of mortality in patients with COPD [14]-[16]. The purpose of this study was to determine if hyperinflation is more closely associated with quality of life, functional status, and pulmonary exacerbations than FEV1 in patients with CF. We used bivariate correlations and backwards regression analysis to assess possible associations between FEV1, lung hyperinflation, and measures of disease severity including questionnaire based quality of life, pulmonary exacerbation frequency, and mortality. Conclusions: FEV1 and lung hyperinflation-as measured by IC and FRC/TLC-are both associated with pulmonary exacerbation frequency. This suggests that chronic dynamic hyperinflation contributes significantly to disease severity in adult cystic fibrosis
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