BACKGROUND: Median survival for cystic fibrosis (CF) patients in Europe is unknown and is likely to be influenced by socioeconomic factors. Using the European CF Society Patient Registry (ECFSPR), median survival estimates were obtained for CF patients across Europe and the impact of socioeconomic status on survival was examined. METHODS: CF subjects known to be alive and in the ECFSPR between 2010 and 2014 were included. Survival curves were estimated using the Kaplan-Meier method. Differences in the survival curves were assessed using the log-rank test. Cox regression was used to estimate the association between socioeconomic factors and the age-specific hazard of death, with adjustment for sex, age at diagnosis, CF transmembrane conductance regulator (CFTR) genotype and transplant status. RESULTS: The final analysis included 13 countries with 31 987 subjects (135 833 person-years of follow-up) and 1435 deaths. Median survival age for these patients in the ECFSPR was 51.7 (95% CI 50.0-53.4) years. After adjusting for potential confounders age at diagnosis, sex, CFTR genotype and transplant status, there remained strong evidence of an association between socioeconomic factors and mortality (p<0.001). Countries in the highest third of healthcare spending had a 46% lower hazard of mortality (HR 0.54, 95% CI 0.45-0.64) than countries in the lowest third of healthcare spending. CONCLUSIONS: Median survival for patients with CF in Europe is comparable to that reported in other jurisdictions and differs by socioeconomic factors.

Cystic Fibrosis Registry of Ireland Dublin Ireland

Dept of Biology and Medical Genetics Faculty of Medicine of Charles University and Motol University Hospital Prague Czechia

Dept of Clinical Sciences and Community Health Laboratory of Medical Statistics Epidemiology and Biometry G A Maccacaro University of Milan Milan Italy

Dept of Paediatrics Justus Liebig University Giessen Giessen Germany

European Cystic Fibrosis Society Patient Registry Karup Denmark

London School of Hygiene and Tropical Medicine London UK

Royal Brompton Hospital and National Heart and Lung Institute Imperial College London London UK

St Vincent's University Hospital and University College Dublin School of Medicine Dublin Ireland

University Hospital Leuven Leuven Belgium

Vaincre La Mucoviscidose Paris France

Eur Respir J. 2021 Oct 1;58(3):2101487. doi: 10.1183/13993003.01487-2021. PubMed

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