The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis
The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis
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DOI：
10.1186/s12931-019-1271-z
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发表时间：
2020-01-08
影响因子：
5.8
通讯作者：
Petkovic, Tatjana Radjenovic
中科院分区：
文献类型：
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作者：
Tran, Tanja;Sterclova, Martina;Petkovic, Tatjana Radjenovic
为了更好地了解特发性肺纤维化（IPF）的自然史，已经建立了几个特发性肺纤维化（IPF）登记处，尽管其规模和随访时间有限。在这里，我们描述了大型欧洲多伙伴IPF登记研究（EMPIRE），并验证了IPF长期生存的预测因素。方法自2014年以来，多国前瞻性EMPIRE登记研究从10个中欧和东欧国家的48个研究中心招募了IPF患者。估计从IPF诊断至死亡的生存期，解释左截断。使用考克斯比例风险回归模型估计预后因素的死亡校正风险比（HR），使用限制性三次样条拟合连续因素。结果该队列包括1620例患者（诊断时平均年龄67.6岁，71%为男性，63%有吸烟史），其中75%在诊断后6个月内入组。中位生存期为4.5年，45%的患者在诊断后5年存活。与GAP I期相比，GAP II期（HR 2.9; 95% CI：2.3-3.7）和III期（HR 4.0; 95% CI：2.8-5.7）的死亡率更高，而在重新定义的临界值下，相应的HR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）的死亡率较高。结论：EMPIRE是IPF患者最大的长期登记研究之一，可更准确地确认长期5年死亡率的预后因素和合并症。它还表明，一些微调的死亡率指标可能会提供一个更准确的长期预后的这些患者。
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.