BACKGROUND Several registries of idiopathic pulmonary fibrosis (IPF) have been established to better understand its natural history, though their size and duration of follow-up are limited. Here, we describe the large European MultiPartner IPF Registry (EMPIRE) and validate predictors of long-term survival in IPF. METHODS The multinational prospective EMPIRE registry enrolled IPF patients from 48 sites in 10 Central and Eastern European countries since 2014. Survival from IPF diagnosis until death was estimated, accounting for left-truncation. The Cox proportional hazards regression model was used to estimate adjusted hazard ratios (HR) of death for prognostic factors, using restricted cubic splines to fit continuous factors. RESULTS The cohort included 1620 patients (mean age at diagnosis 67.6 years, 71% male, 63% smoking history), including 75% enrolled within 6 months of diagnosis. Median survival was 4.5 years, with 45% surviving 5 years post-diagnosis. Compared with GAP stage I, mortality was higher with GAP stages II (HR 2.9; 95% CI: 2.3-3.7) and III (HR 4.0; 95% CI: 2.8-5.7) while, with redefined cut-offs, the corresponding HRs were 2.7 (95% CI: 1.8-4.0) and 5.8 (95% CI: 4.0-8.3) respectively. Mortality was higher with concurrent pulmonary hypertension (HR 2.0; 95% CI: 1.5-2.9) and lung cancer (HR 2.6; 95% CI: 1.3-4.9). CONCLUSIONS EMPIRE, one of the largest long-term registries of patients with IPF, provides a more accurate confirmation of prognostic factors and co-morbidities on longer term five-year mortality. It also suggests that some fine-tuning of the indices for mortality may provide a more accurate long-term prognostic profile for these patients.

中文翻译：

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欧洲MultiPartner IPF注册中心（EMPIRE）：验证特发性肺纤维化的长期预后因素。

背景技术已建立了一些特发性肺纤维化（IPF）登记册，以更好地了解其自然病史，尽管其规模和随访时间有限。在这里，我们描述了大型的欧洲MultiPartner IPF注册中心（EMPIRE），并验证了IPF中长期生存的预测指标。方法自2014年以来，多国性前瞻性EMPIRE注册中心从10个中欧和东欧国家的48个地点招募了IPF患者。从IPF诊断到死亡的估计生存时间，考虑了左截短。使用Cox比例风险回归模型，通过使用受限三次样条拟合连续因素来估计预后因素的调整后死亡危险比（HR）。结果该队列包括1620名患者（诊断时平均年龄67.6岁，男性71％，吸烟史63％），其中包括75％的患者在诊断后6个月内入选。中位生存期为4.5年，诊断后5年生存率为45％。与第一阶段GAP相比，第二阶段GAP（HR 2.9; 95％CI：2.3-3.7）和III阶段（HR 4.0; 95％CI：2.8-5.7）的死亡率更高，而重新定义的临界值是相应的HRs分别为2.7（95％CI：1.8-4.0）和5.8（95％CI：4.0-8.3）。并发肺动脉高压（HR 2.0; 95％CI：1.5-2.9）和肺癌（HR 2.6; 95％CI：1.3-4.9）的死亡率更高。结论EMPIRE是IPF患者最大的长期注册资料之一，可以更准确地确定长期5年死亡率的预后因素和合并症。这也表明死亡率指标的一些微调可以为这些患者提供更准确的长期预后情况。