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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,

. 2020 ; 21 (1) : 11. [pub] 20200108

Language English Country Great Britain

Document type Journal Article, Multicenter Study

Persistent link   https://www.medvik.cz/link/bmc20028612

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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$a Tran, Tanja $u Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada. Centre for Clinical Epidemiology, Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada.
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$a 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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$a Šterclová, Martina $u Department of Respiratory Medicine of the First Faculty of Medicine Charles University, Thomayer Hospital, Vídeňská 800, 140 59, Prague 4, Czech Republic.
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$a Mogulkoc, Nesrin $u Department of Chest Diseases, Faculty of Medicine, Ege University, Izmir, Turkey.
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$a Lewandowska, Katarzyna $u 1st Department of Pulmonary Diseases, Institute of Tuberculosis and Lung Diseases, Warsaw, Poland.
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$a Müller, Veronika $u Department of Pulmonology, Faculty of Medicine, Semmelweis University, Budapest, Hungary.
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$a Hájková, Marta $u Clinic of Pneumology and Phthisiology, University Hospital Bratislava, Bratislava, Slovakia.
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$a Kramer, Mordechai R $u Institute of Pulmonary Medicine, Rabin Medical Center, Petah Tikva, Israel.
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$a Jovanović, Dragana $u University Hospital of Pulmonology, Clinical Center of Serbia, Belgrade, Serbia.
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$a Tekavec-Trkanjec, Jasna $u Department of Pulmonology, Clinical Hospital Dubrava, Zagreb, Croatia.
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$a Studnicka, Michael $u Clinical Research Center Salzburg, Salzburg, Austria.
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$a Stoeva, Natalia $u Department of Pulmonology, Acibadem City Clinic Tokuda Hospital, Sofia, Bulgaria.
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$a Hejduk, Karel $u Institute of Biostatistics and Analyses, Faculty of Medicine, Masaryk University, Brno, Czech Republic.
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$a Dušek, Ladislav $u Institute of Biostatistics and Analyses, Faculty of Medicine, Masaryk University, Brno, Czech Republic.
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$a Suissa, Samy $u Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada. Centre for Clinical Epidemiology, Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada.
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$a Vašáková, Martina $u 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.
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