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

Tanja Tran, Martina Šterclová, Nesrin Mogulkoc, Katarzyna Lewandowska, Veronika Müller, Marta Hájková, Mordechai R. Kramer, Dragana Jovanović, Jasna Tekavec-Trkanjec, Michael Studnicka, Natalia Stoeva, Karel Hejduk, Ladislav Dušek, Samy Suissa, Martina Vašáková, Beata Zolnowska, Vladimír Bartoš, Hradec Králové, Robert Slivka, Ladislav LacinaMartina Doubková, Radka Bittenglová, Magdalena Martusewicz-Boros, Monika Žurková, Imrich Jonner, Amelia Szymanowska-Narloch, Małgorzata Sobiecka, Vladimíra Lošťáková, Marzena Trzaska-Sobczak, Richard Tyl, Pawel Sliwinski, Miklós Zsiray, Anikó Bohács, Sebastian Majewski, Hana Šuldová, Bohumil Matula, Lenka Šišková, Ján Plutinský, Ana Jakić, Štefan Tóth, Zoltán Balikó, Margita Bučeková, Jana Pšíkalová, Tomasz Stachura, František Petřík, Jan Anton, Jaroslav Lněnička, Marina Roksandić Milenkovic, Imre Lajkó, Vladimír Řihák, Zsuzsanna Szalai, Paulina Jurek, Aleksander Kania, Štefan Laššán, Tatjana Pejcic, Pavel Reiterer, Lukasz Borucki, Renata Králová, Pavlína Musilová, Tomáš Snížek, Daniel Doležal, Jiří Homolka, Hana Hortvíková, Suzana Mladinov, Peter Palúch, Roman Hrdina, Maria Szilasi, Violeta Vučinić-Mihailović, Róbert Vyšehradský, Radka Mokošová, Agata Nowicka, Tatjana Radjenovic Petkovic

Research output: Contribution to journalArticle

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.

Original languageEnglish
Article number11
JournalRespiratory Research
Volume21
Issue number1
DOIs
Publication statusPublished - Jan 8 2020

    Fingerprint

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Tran, T., Šterclová, M., Mogulkoc, N., Lewandowska, K., Müller, V., Hájková, M., Kramer, M. R., Jovanović, D., Tekavec-Trkanjec, J., Studnicka, M., Stoeva, N., Hejduk, K., Dušek, L., Suissa, S., Vašáková, M., Zolnowska, B., Bartoš, V., Králové, H., Slivka, R., ... Petkovic, T. R. (2020). The European MultiPartner IPF registry (EMPIRE): Validating long-term prognostic factors in idiopathic pulmonary fibrosis. Respiratory Research, 21(1), [11]. https://doi.org/10.1186/s12931-019-1271-z