A role for cardiopulmonary exercise testing in detecting physiological changes underlying health status in Idiopathic pulmonary fibrosis: a feasibility study.

INTRODUCTION There is limited data available on the use of CPET as a predictive tool for disease outcomes in the setting of IPF. We investigated the feasibility of undertaking CPET and the relationship between CPET and quality of life measurements in a well-defined population of mild and moderate IPF patients. METHODS A prospective, single-centre observational study. RESULTS Thirty-two IPF patients (mild n = 23, moderate n = 9) participated in the study, n = 13 mild patients attended for repeat CPET testing at 12 months. At baseline, total K-BILD scores and total IPF-PROM scores significantly correlated with 6MWT distance, but not with baseline FVC % predicted, TLco % predicted, baseline or minimum SpO2. VO2 peak/kg at AT positively correlated with total scores, breathlessness/activity and chest domains of the K-BILD questionnaire (p < 0.05). VO2 peak significantly correlated with total IPF PROM scores and wellbeing domains (p < 0.05), with a trend towards statistical significance for total IPF-PROM and VO2 peak/kg at anaerobic threshold (p = 0.06). There was a statistically significant reduction in FVC% predicted at 12 months follow up, although the mean absolute decline was < 10% (p < 0.05). During this period VO2 peak significantly reduced (21.6 ml/kg/min ± 2.9 vs 19.1 ± 2.8; p = 0.017), with corresponding reductions in total K-BILD and breathlessness/activity domains that exceeded the MCID for responsiveness. Lower baseline VO2 peak/kg at anaerobic threshold correlated with greater declines in total K-BILD scores (r =  - 0.62, 0.024) at 12 months. Whilst baseline FVC% predicted or TLco % predicted did not predict change in health status, CONCLUSION: We have shown that it is feasible to undertake CPET in patients with mild to moderate IPF. CPET measures of VO2 peak correlated with both baseline and change in K-BILD measurements at 1 year, despite relatively stable standard lung function (declines of < 10% in FVC), suggesting its potential sensitivity to detect physiological changes underlying health status.