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Articles
Articles
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Source: BMČ - články
Title
The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis / T. Tran, M. Šterclová, N. Mogulkoc, K. Lewandowska, V. Müller, M. Hájková, MR. Kramer, D. Jovanović, J. Tekavec-Trkanjec, M. Studnicka, N. Stoeva, K. Hejduk, L. Dušek, S. Suissa, M. Vašáková, EMPIRE registry,
Author
Tran, Tanja
Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada. Centre for Clinical Epidemiology, Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada.

Šterclová, Martina
Department of Respiratory Medicine of the First Faculty of Medicine Charles University, Thomayer Hospital, Vídeňská 800, 140 59, Prague 4, Czech Republic.

Mogulkoc, Nesrin
Department of Chest Diseases, Faculty of Medicine, Ege University, Izmir, Turkey.

Lewandowska, Katarzyna
1st Department of Pulmonary Diseases, Institute of Tuberculosis and Lung Diseases, Warsaw, Poland.

Müller, Veronika
Department of Pulmonology, Faculty of Medicine, Semmelweis University, Budapest, Hungary.

Hájková, Marta
Clinic of Pneumology and Phthisiology, University Hospital Bratislava, Bratislava, Slovakia.

Kramer, Mordechai R
Institute of Pulmonary Medicine, Rabin Medical Center, Petah Tikva, Israel.

Jovanovic, Dragana
University Hospital of Pulmonology, Clinical Center of Serbia, Belgrade, Serbia.

Tekavec-Trkanjec, Jasna
Department of Pulmonology, Clinical Hospital Dubrava, Zagreb, Croatia.

Studnicka, Michael
Clinical Research Center Salzburg, Salzburg, Austria.

Stoeva, Natalia
Department of Pulmonology, Acibadem City Clinic Tokuda Hospital, Sofia, Bulgaria.

Hejduk, Karel
Institute of Biostatistics and Analyses, Faculty of Medicine, Masaryk University, Brno, Czech Republic.

Dušek, Ladislav
Institute of Biostatistics and Analyses, Faculty of Medicine, Masaryk University, Brno, Czech Republic.

Suissa, Samy
Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada. Centre for Clinical Epidemiology, Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada.

Vašáková, Martina
Department of Respiratory Medicine of the First Faculty of Medicine Charles University, Thomayer Hospital, Vídeňská 800, 140 59, Prague 4, Czech Republic. martina.vasakova@ftn.cz.

EMPIRE registry
Cited source
Respiratory research. 2020, roč. 21, č. 1, s. 11. ISSN: 1465-9921; 1465-993X (elektronická verze).
Date of issue
2020
Language
English
Country
United Kingdom
Document type
Articles
DOI
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Link
Record number
bmc20028612
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English 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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