Lung clearance index trajectories and survival in people with cystic fibrosis

Hintergrund Progressive muco-obstructive lung disease leads to premature death in people with Cystic Fibrosis (pwCF). Lung clearance index (LCI), a biomarker of ventilation inhomogeneity in peripheral airways, may predict survival in pwCF. We aim to examine the association between LCI trajectory patterns and risk of death or lung transplantation (LTx) in pwCF.

Methoden We performed a retrospective, longitudinal analysis in a cohort of pwCF aged >5 years and at least one LCI measurement. LCI was measured using a nitrogen multiple-breath washout setup during clinical routine visits in Bern (Switzerland) between 1986 and 2006. Preliminary analyses include descriptive statistics. We aim to apply latent classes analysis (LCA) to serial FEV1 and LCI measurements and link these classes to the compound outcome risk of death or LTx in pwCF until 12/2018.

Ergebnisse In total, 268 pwCF were eligible and 259 (135 females) were included. Mean (SD) age was 15.7 (0.9) years at study entry. PwCF contributed (median, IQR) 11 (5-19) observations per person including 2787 LCI measurements. Median LCI was 18.4 (IQR = 13.8-24.6) units. LCA models with the fewest number of classes that best fit the data remain to be selected. In 106 (40.9%) pwCF the primary outcome occurred: 59 patients received LTX and 47 died.

Schlussfolgerung Our preliminary analyses suggest that the dataset is correctly setup and sufficiently large for LCA. We will explore if pwCF attributed to specific LCI clusters were more likely to die or receive LTX in this study.

Publication History

Article published online:
22 February 2024