Abstract
Background Accelerated lung function decline in individuals with cystic fibrosis (CF) starts in adolescence with respiratory complications being the most common cause of death in later life. Factors contributing to lung function decline are not well understood, in particular its relationship with structural lung disease in early childhood. Detection and management of structural lung disease could be an important step in improving outcomes in CF patients.
Methods Annual chest computed tomography (CT) scans were available from 2005 to 2016 as a part of the AREST CF cohort for children aged 3 months to 6 years. Annual spirometry measurements were available for 89.77% of the cohort (167 children aged 5–6 years) from age 5 to 15 years through outpatient clinics at Perth Children's Hospital (Perth, Australia) and The Royal Children's Hospital in Melbourne (Melbourne, Australia) (697 measurements, mean±sd age 9.3±2.1 years).
Results Children with a total CT score above the median at age 5–6 years were more likely to have abnormal forced expiratory volume in 1 s (FEV1) (adjusted hazard ratio 2.67 (1.06–6.72), p=0.037) during the next 10 years compared to those below the median chest CT score. The extent of all structural abnormalities except bronchial wall thickening were associated with lower FEV1 Z-scores. Mucus plugging and trapped air were the most predictive sub-score (adjusted mean change −0.17 (−0.26 – −0.07) p<0.001 and −0.09 (−0.14 – −0.04) p<0.001, respectively).
Discussion Chest CT identifies children at an early age who have adverse long-term outcomes. The prevention of structural lung damage should be a goal of early intervention and can be usefully assessed with chest CT. In an era of therapeutics that might alter disease trajectories, chest CT could provide an early readout of likely long-term success.
Abstract
Structural lung changes identified on a chest CT scan in children with cystic fibrosis under 6 years of age can identify those at risk of adverse long-term outcomes http://bit.ly/39QH5jv
Footnotes
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Conflict of interest: L. Turkovic has nothing to disclose.
Conflict of interest: D. Caudri has nothing to disclose.
Conflict of interest: T. Rosenow reports grants from National Health and Medical Research Council, during the conduct of the study. In addition, Dr. Rosenow has a patent PCT/AU2016/000079 issued to the Australian Patent Office.
Conflict of interest: O. Breuer has nothing to disclose.
Conflict of interest: C. Murray has nothing to disclose.
Conflict of interest: H.A.W.M. Tiddens reports other from Roche and Novartis, grants from CFF, Vertex, Chiesi, Vectura and NCFS, outside the submitted work. In addition, he has a patent PRAGMA-CF scoring system issued and is head of the Erasmus MC-Sophia Children's Hospital core laboratory LungAnalysis.
Conflict of interest: F. Ramanauskas has nothing to disclose.
Conflict of interest: S.C. Ranganathan has nothing to disclose.
Conflict of interest: G.L. Hall has nothing to disclose.
Conflict of interest: S.M. Stick has nothing to disclose.
Support statement: Cystic Fibrosis Foundation (http://dx.doi.org/10.13039/100000897), National Health and Medical Research Council (http://dx.doi.org/10.13039/501100000925). Funding information for this article has been deposited with the Crossref Funder Registry.
- Received April 16, 2019.
- Accepted February 12, 2020.
- Copyright ©ERS 2020