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Multicenter Study
. 2020 Jan 8;21(1):11.
doi: 10.1186/s12931-019-1271-z.

The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

Tanja Tran  1   2 , Martina Šterclová  3 , Nesrin Mogulkoc  4 , Katarzyna Lewandowska  5 , Veronika Müller  6 , Marta Hájková  7 , Mordechai R Kramer  8 , Dragana Jovanović  9 , Jasna Tekavec-Trkanjec  10 , Michael Studnicka  11 , Natalia Stoeva  12 , Karel Hejduk  13 , Ladislav Dušek  13 , Samy Suissa  1   2 , Martina Vašáková  14 ; EMPIRE registry
Collaborators, Affiliations
Multicenter Study

The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

Tanja Tran et al. Respir Res. .

Abstract

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.

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Conflict of interest statement

S.S. has received research grants from Boehringer Ingelheim and Novartis and has participated in advisory board meetings or as speaker for AstraZeneca, Boehringer-Ingelheim, and Novartis. L.D. and K.H. have received grants from Boehringer-Ingelheim and Roche. J.T.-T. has received support from Boehringer-Ingelheim and Roche. None of the other authors have any competing interests to disclose.

Figures

Fig. 1
Fig. 1
Smooth cubic spline curve of the adjusted hazard ratio (solid line) and 95% CIs (dotted lines) of death, estimated by Cox proportional hazards model, as a function of: a) age at IPF diagnosis; b) % predicted FVC; and c) % predicted TLCO
Fig. 2
Fig. 2
Kaplan–Meier survival functions for the cohort of 1620 patients with IPF from the time of their IPF diagnosis over a 5-year follow-up period, according to the a) original GAP stage classification and b) with redefined cut-points
See this image and copyright information in PMC

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