Abstract
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.
Abstract
Median survival for patients with CF in Europe is similar to that reported in other jurisdictions and differs depending on socioeconomic status, with measures of higher healthcare spending associated with improved survival. https://bit.ly/3jYF37q
Footnotes
This article has supplementary material available from erj.ersjournals.com
This article has an editorial commentary: https://doi.org/10.1183/13993003.01487-2021
Author contributions: E.F. McKone had full access to all the data and the final responsibility for the decision to publish. All authors were involved in data collection or the study design as well as manuscript preparation and review.
Conflict of interest: E.F. McKone reports grants and personal fees from Vertex Pharmaceuticals, personal fees from Novartis, nonfinancial support from A Menarini, and grants from Gilead, outside the submitted work.
Conflict of interest: C. Ariti has nothing to disclose.
Conflict of interest: A. Jackson has nothing to disclose.
Conflict of interest: A. Zolin has nothing to disclose.
Conflict of interest: S.B. Carr reports nonfinancial support and other from Chiesi Pharmaceuticals (Advisory Board fee), nonfinancial support and other from Vertex Pharmaceuticals (Advisory Board, lecture fee, travel, Steering Committee), other from Zambon Pharmaceuticals (Advisory Board fee), other from Insmed (Advisory Board fee), outside the submitted work.
Conflict of interest: A. Orenti has nothing to disclose.
Conflict of interest: J.G. van Rens has nothing to disclose.
Conflict of interest: L. Lemonnier has nothing to disclose.
Conflict of interest: M. Macek Jr has nothing to disclose.
Conflict of interest: R.H. Keogh has nothing to disclose.
Conflict of interest: L. Naehrlich reports that he has received institutional fees for site participation in clinical trials from Vertex Pharmaceuticals.
Support statement: There was no external funding to the ECFSPR for this study. Statistical analysis was funded through a grant from the ECFSPR to London School of Hygiene and Tropical Medicine. Funding information for this article has been deposited with the Crossref Funder Registry.
- Received June 12, 2020.
- Accepted February 12, 2021.
- Copyright ©The authors 2021. For reproduction rights and permissions contact permissions{at}ersnet.org
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