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.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.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).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.
