PT - JOURNAL ARTICLE AU - Naomi E. Wijker AU - Suzanna Vidmar AU - Keith Grimwood AU - Peter D. Sly AU - Catherine A. Byrnes AU - John B. Carlin AU - Peter J. Cooper AU - Colin F. Robertson AU - R. John Massie AU - Mariette P.C. Kemner van de Corput AU - Joyce Cheney AU - Harm A.W.M. Tiddens AU - Claire E. Wainwright ED - , TI - Early Markers of Cystic Fibrosis Structural Lung Disease: Follow-Up of the ACFBAL Cohort AID - 10.1183/13993003.01694-2019 DP - 2020 Jan 01 TA - European Respiratory Journal PG - 1901694 4099 - http://erj.ersjournals.com/content/early/2020/01/03/13993003.01694-2019.short 4100 - http://erj.ersjournals.com/content/early/2020/01/03/13993003.01694-2019.full AB - Introduction Little is known about early predictors of later cystic fibrosis (CF) structural lung disease. This study examined early predictors of progressive structural lung abnormalities in children who completed the Australasian CF Bronchoalveolar Lavage (ACFBAL) clinical trial at age 5-years and participated in an observational follow-up study (CF-FAB).Methods Eight Australian and New Zealand CF centres participated in CF-FAB and provided follow-up chest computed-tomography (CT) scans for children who had completed the ACFBAL study with baseline scans at age 5-years. CT-scans were annotated using PRAGMA-CF scoring. Ordinal regression analysis and linear regression were used to investigate associations between PRAGMA-CF outcomes at follow-up and variables measured during the ACFBAL study.Results Ninety-nine of 157 ACFBAL children (mean age 13-years, standard deviation 1.5) participated in the CF-FAB study. The probability of bronchiectasis at follow-up increased with airway disease severity on the baseline CT-scan. In multiple regression (retaining factors at p<0.05) the extent of bronchiectasis at follow-up was associated with baseline atelectasis (odds ratio (OR)7.2; 95% confidence interval (CI) 2.4, 22; P≤ 0.001), bronchoalveolar lavage (BAL) log2 interleukin (IL)-8 (OR 1.2 95%CI 1.05, 1.5; p=0.010) and body mass index z-score (OR 0.49, 95%CI 0.24, 1.00; p=0.05) at age 5-years. Percent trapped air at follow-up was associated with BAL log2 IL-8 (coefficient 1.3, 95%CI 0.57, 2.1; p<0.001) at age 5-years.Conclusions The extent of airway disease, atelectasis, airway inflammation, and poor nutritional status in early childhood are risk-factors for progressive structural lung disease in adolescence.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Wijker reports grants from Australian National Health and Medical Research Council, grants from Australian National Health and Medical Research Council, during the conduct of the study.Conflict of interest: Ms. Vidmar reports grants from Australian National Health and Medical Research Council, grants from Australian National Health and Medical Research Council, during the conduct of the study.Conflict of interest: Dr. Grimwood reports grants from the Australian National Health and Medical Research Council during the conduct of the studyConflict of interest: Dr. Sly reports grants from Australian National Health and Medical Research Council, grants from Australian National Health and Medical Research Council, during the conduct of the study.Conflict of interest: Dr. Byrnes reports grants from Australian National Health and Medical Research Council, grants from Australian National Health and Medical Research Council, grants from Faculty Research Development Fund, University of Auckland, New Zealand, during the conduct of the study.Conflict of interest: Dr. Carlin reports grants from Australian National Health and Medical Research Council, grants from Australian National Health and Medical Research Council, during the conduct of the study.Conflict of interest: Dr. Cooper reports grants from Australian National Health and Medical Research Council, grants from Australian National Health and Medical Research Council, during the conduct of the study.Conflict of interest: Dr. Robertson reports grants from Australian National Health and Medical Research Council, grants from Australian National Health and Medical Research Council, during the conduct of the study.Conflict of interest: Dr. Massie reports grants from Australian National Health and Medical Research Council, during the conduct of the study.Conflict of interest: Dr. Kemner-van de Corput reports grants from Australian National Health and Medical Research Council, grants from Australian National Health and Medical Research Council, during the conduct of the study.Conflict of interest: Dr. Cheney reports grants from Australian National Health and Medical Research Council, grants from Australian National Health and Medical Research Council, during the conduct of the study.Conflict of interest: Dr. Tiddens reports grants from Australian National Health and Medical Research Council, grants from Australian National Health and Medical Research Council, during the conduct of the study; other from Roche, other from Novartis, grants from CFF, grants from Vertex, grants from Gilead, grants from Chiesi, grants from Vectura, outside the submitted work; In addition, Dr. Tiddens has a patent PRAGMA-CF scoring system with royalties paid and I am heading the Erasmus MC-Sophia Children's Hospital core laboratory Lung Analysis.Conflict of interest: Dr. Wainwright reports grants from Australian National Health and Medical Research Council, grants from Australian National Health and Medical Research Council, during the conduct of the study; and Income on a per patient basis derived from Pharmaceutical Studies - Vertex Pharmaceuticals Inc., & Boehringer-Ingelheim.