PT - JOURNAL ARTICLE AU - Leonie Tepper AU - Elisabeth Utens AU - Daan Caudri AU - Alexandra Quittner AU - Harm Tiddens TI - Tracking disease progression in cystic fibrosis using bronchiectasis, trapped air and quality of life DP - 2012 Sep 01 TA - European Respiratory Journal PG - P1450 VI - 40 IP - Suppl 56 4099 - http://erj.ersjournals.com/content/40/Suppl_56/P1450.short 4100 - http://erj.ersjournals.com/content/40/Suppl_56/P1450.full SO - Eur Respir J2012 Sep 01; 40 AB - Background. Progression of cystic fibrosis (CF) is characterized by bronchiectasis (BE) and trapped air (TA) on CT. We hypothesize that progression of BE, and TA results in a lower Health Related Quality of Life (HRQoL), assessed by the Cystic Fibrosis Questionnaire-Revised (CFQ-R).Objective. To evaluate associations between changes in BE, TA, and CFQ-R over time.Methods. Cohort study (July 2007-January 2012). Clinical stable CF patients, with two routine bi-annual chest CTs, and CFQ-Rs. CT scans were anonymous and randomly scored, using CF-CT BE and TA scores. Scores are expressed as % of maximum score. CFQ-R was completed by children (aged 6-13 years) and adolescents (aged ≥ 14 years). Score-range 0-100, higher scores indicate better HRQoL. For changes in CF-CT BE, TA score, CFQ-R respiratory-symptoms domain scores, their correlations, and to test the hypothesis we used Student's paired t-test, Spearman's correlation coefficient, and linear regression model, adjusted for age and gender.Results. CF patients (n= 40): mean age T1 11.9 years, T2 13.8 years. In two years there was a significant increase in CF-CT BE scores (p=0.03) and CF-CT TA scores (p=0.03), but not in CFQ-R scores. At T1 CF-CT BE scores (p<0.01, r=-0.49) and CF-CT TA scores (p=0.04, r=-0.34) correlate with CFQ-R scores. At T2 similar correlations were found: CF-CT BE (p<0.01, r=-0.41), CF-CT TA (p=0.02, r=-0.37). Change in CF-CT BE and TA scores did not correlate to change in CFQ-R scores.Conclusion. BE and TA correlates to HRQoL. Modest progression of BE and TA did not result in impairment of HRQoL.Supported by an unconditional grant by Gilead Sciences, Inc and the Sophia fund: “steun door Zeevaart.”