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 Cystic Fibrosis 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 (KM) 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, CFTR genotype and transplant status.
Findings 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 years (95% C.I. 50.0–53.4). 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 with higher health care spending had a 46% lower hazard of mortality (HR: 0.54, 95% CI: 0.45–0.64) than countries with lowest health care spending.
Interpretation Median survival for patients with CF in Europe is comparable to that reported in other jurisdictions and differs by socioeconomic factors.
Footnotes
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Conflict of interest: Dr. McKone reports grants and personal fees from Vertex Pharmaceutocals, personal fees from Novartis, non-financial support from A Menarini, grants from Gilead, outside the submitted work.
Conflict of interest: Dr. Ariti has nothing to disclose.
Conflict of interest: Dr. Jackson has nothing to disclose.
Conflict of interest: Dr. Zolin has nothing to disclose.
Conflict of interest: Dr. Carr reports non-financial support and other from Chiesi Pharmaceuticals, non-financial support and other from Vertex Pharmaceuticals, other from Zambon Pharmaceuticals, other from Insmed, outside the submitted work.
Conflict of interest: Dr. Orenti has nothing to disclose.
Conflict of interest: Dr. van Rens has nothing to disclose.
Conflict of interest: Dr. LEMONNIER has nothing to disclose.
Conflict of interest: Dr. Macek has nothing to disclose.
Conflict of interest: Dr. Keogh has nothing to disclose.
Conflict of interest: Dr. Naehrlich reports that he has received institutional fees for site participation in clinical trials from Vertex Pharmaceuticals.
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- Received June 12, 2020.
- Accepted February 12, 2021.
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