TY - JOUR
T1 - Survival estimates in European cystic fibrosis patients and the impact of socioeconomic factors
T2 - a retrospective registry cohort study
AU - McKone, Edward F.
AU - Ariti, Cono
AU - Jackson, Abaigeal
AU - Zolin, Anna
AU - Carr, Siobhán B.
AU - Orenti, Annalisa
AU - van Rens, Jacqui G.
AU - Lemonnier, Lydie
AU - Jra, Milan Macek
AU - Keogh, Ruth H.
AU - Naehrlich, Lutz
AU - The European Cystic Fibrosis Society Patient Registry
A2 - Aleksejeva, E.
A2 - Bardin, E.
A2 - Burgel, P. R.
A2 - Cosgriff, R.
A2 - Daneau, G.
A2 - de Monestrol, I.
A2 - Drevinek, P.
A2 - Fletcher, G.
A2 - Fustik, S.
A2 - Gulmans, V.
A2 - Hatziagorou, E.
A2 - Jung, A.
A2 - Kashirskaya, N.
A2 - Kayserova, H.
A2 - Krivec, U.
A2 - Lindblad, A.
A2 - Makukh, H.
A2 - Malakauskas, K.
A2 - Mei-Zahev, M.
A2 - Olesen, H. V.
A2 - Padoan, R.
A2 - Párniczky, A.
A2 - Pastor-Vivero, M. D.
A2 - Pereira, L.
A2 - Pfleger, A.
A2 - Pop, L.
A2 - Rodic, M.
A2 - Turcu, O.
N1 - Funding Information:
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.
Funding Information:
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. C. Ariti has nothing to disclose. A. Jackson has nothing to disclose. A. Zolin has nothing to disclose. 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. A. Orenti has nothing to disclose. J.G. van Rens has nothing to disclose. L. Lemonnier has nothing to disclose. M. Macek Jr has nothing to disclose. R.H. Keogh has nothing to disclose. L. Naehrlich reports that he has received institutional fees for site participation in clinical trials from Vertex Pharmaceuticals.
Publisher Copyright:
© The authors 2021.
PY - 2021/9/1
Y1 - 2021/9/1
N2 - 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 31987 subjects (135833 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.
AB - 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 31987 subjects (135833 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.
UR - http://www.scopus.com/inward/record.url?scp=85116497536&partnerID=8YFLogxK
U2 - 10.1183/13993003.02288-2020
DO - 10.1183/13993003.02288-2020
M3 - Article
C2 - 33678607
AN - SCOPUS:85116497536
SN - 0903-1936
VL - 58
JO - European Respiratory Journal
JF - European Respiratory Journal
IS - 3
M1 - 2002288
ER -