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. 2021 Oct 1;58(3):2002288.
doi: 10.1183/13993003.02288-2020. Print 2021 Sep.

Survival estimates in European cystic fibrosis patients and the impact of socioeconomic factors: a retrospective registry cohort study

Edward F McKone  1 , Cono Ariti  2 , Abaigeal Jackson  3 , Anna Zolin  4 , Siobhán B Carr  5 , Annalisa Orenti  4 , Jacqui G van Rens  6   7 , Lydie Lemonnier  8 , Milan Macek Jr  9 , Ruth H Keogh  2 , Lutz Naehrlich  10 ; European Cystic Fibrosis Society Patient Registry; ECFSPR contributors list consists of the representatives of the countries whose data is used in this article, and the members of the Scientific Committee who reviewed the initial data application and the final manuscript. Collaborating authors:
Collaborators, Affiliations
Free article

Survival estimates in European cystic fibrosis patients and the impact of socioeconomic factors: a retrospective registry cohort study

Edward F McKone et al. Eur Respir J. .
Free article

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.

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Conflict of interest statement

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.

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