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

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F61989592%3A15110%2F20%3A73600848" target="_blank" >RIV/61989592:15110/20:73600848 - isvavai.cz</a>

Alternative codes found

RIV/00216224:14110/20:00115999 RIV/00064211:_____/20:W0000037

Result on the web

<a href="https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-019-1271-z" target="_blank" >https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-019-1271-z</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1186/s12931-019-1271-z" target="_blank" >10.1186/s12931-019-1271-z</a>

Result language

angličtina

Original language name

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

Original language description

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.

Czech name

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Czech description

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Type

J_SC - Article in a specialist periodical, which is included in the SCOPUS database

CEP classification

—

OECD FORD branch

30203 - Respiratory systems

Project

—

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2020

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Respiratory Research

ISSN

1465-9921

e-ISSN

—

Volume of the periodical

21

Issue of the periodical within the volume

1

Country of publishing house

GB - UNITED KINGDOM

Number of pages

9

Pages from-to

"'1'"-"'9'"

UT code for WoS article

000512718200003

EID of the result in the Scopus database

2-s2.0-85077714238