PT - JOURNAL ARTICLE AU - Alex R Horsley AU - John Belcher AU - Katie Bayfield AU - Brooke Bianco AU - Steve Cunningham AU - Catherine Fullwood AU - Andrew Jones AU - Anna Shawcross AU - Jaclyn A Smith AU - Anirban Maitra AU - Francis J Gilchrist TI - Longitudinal assessment of lung clearance index to monitor disease progression in children and adults with cystic fibrosis AID - 10.1136/thoraxjnl-2021-216928 DP - 2021 Jul 22 TA - Thorax PG - thoraxjnl-2021-216928 4099 - http://thorax.bmj.com/content/early/2021/07/22/thoraxjnl-2021-216928.short 4100 - http://thorax.bmj.com/content/early/2021/07/22/thoraxjnl-2021-216928.full AB - Background Lung clearance index (LCI) is a valuable research tool in cystic fibrosis (CF) but clinical application has been limited by technical challenges and uncertainty about how to interpret longitudinal change. In order to help inform clinical practice, this study aimed to assess feasibility, repeatability and longitudinal LCI change in children and adults with CF with predominantly mild baseline disease.Methods Prospective, 3-year, multicentre, observational study of repeated LCI measurement at time of clinical review in patients with CF >5 years, delivered using a rapid wash-in system.Results 112 patients completed at least one LCI assessment and 98 (90%) were still under follow-up at study end. The median (IQR) age was 14.7 (8.6–22.2) years and the mean (SD) FEV1 z-score was −1.2 (1.3). Of 81 subjects with normal FEV1 (>−2 z-scores), 63% had raised LCI (indicating worse lung function). For repeat stable measurements within 6 months, the mean (limits of agreement) change in LCI was 0.9% (−18.8% to 20.7%). A latent class growth model analysis identified four discrete clusters with high accuracy, differentiated by baseline LCI and FEV1. Baseline LCI was the strongest factor associated with longitudinal change. The median total test time was under 19 min.Conclusions Most patients with CF with well-preserved lung function show stable LCI over time. Cluster behaviours can be identified and baseline LCI is a risk factor for future progression. These results support the use of LCI in clinical practice in identifying patients at risk of lung function decline.Data are available upon reasonable request to the corresponding author.