St Vincent's University Hospital and University College Dublin School of Medicine, Dublin, Ireland
London School of Hygiene and Tropical Medicine, London, UK.
Eur Respir J. 2021 Oct 1;58(3). doi: 10.1183/13993003.02288-2020. Print 2021 Sep.
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.
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 () genotype and transplant status.
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, 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.
Median survival for patients with CF in Europe is comparable to that reported in other jurisdictions and differs by socioeconomic factors.
欧洲囊性纤维化（CF）患者的中位生存期尚不清楚，可能受到社会经济因素的影响。本研究利用欧洲 CF 学会患者注册（ECFSPR），获得了欧洲 CF 患者的中位生存估计值，并探讨了社会经济状况对生存的影响。
纳入 2010 年至 2014 年间在 ECFSPR 中已知存活的 CF 患者。使用 Kaplan-Meier 方法估计生存曲线。采用对数秩检验比较生存曲线的差异。使用 Cox 回归估计社会经济因素与特定年龄死亡风险之间的关联，调整性别、诊断时年龄、CF 跨膜电导调节因子（CFTR）基因型和移植状态。
最终分析纳入了 13 个国家的 31987 例患者（135833 人年随访）和 1435 例死亡。这些患者在 ECFSPR 的中位生存年龄为 51.7（95%CI 50.0-53.4）岁。调整潜在混杂因素诊断时年龄、性别、CFTR 基因型和移植状态后，社会经济因素与死亡率之间仍存在显著关联（p<0.001）。医疗保健支出最高的三分之一国家的死亡率危险比（HR）为 0.54（95%CI 0.45-0.64），显著低于医疗保健支出最低的三分之一国家。
欧洲 CF 患者的中位生存期与其他司法管辖区报告的结果相当，但因社会经济因素而异。
