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.

1st Department of Pulmonary Diseases Institute of Tuberculosis and Lung Diseases Warsaw Poland

Centre for Clinical Epidemiology Lady Davis Institute for Medical Research Jewish General Hospital Montreal Quebec Canada

Clinic of Pneumology and Phthisiology University Hospital Bratislava Bratislava Slovakia

Clinical Research Center Salzburg Salzburg Austria

Department of Chest Diseases Faculty of Medicine Ege University Izmir Turkey

Department of Epidemiology Biostatistics and Occupational Health McGill University Montreal Quebec Canada

Department of Pulmonology Acibadem City Clinic Tokuda Hospital Sofia Bulgaria

Department of Pulmonology Clinical Hospital Dubrava Zagreb Croatia

Department of Pulmonology Faculty of Medicine Semmelweis University Budapest Hungary

Department of Respiratory Medicine of the 1st Faculty of Medicine Charles University Thomayer Hospital Vídeňská 800 140 59 Prague 4 Czech Republic

Institute of Biostatistics and Analyses Faculty of Medicine Masaryk University Brno Czech Republic

Institute of Pulmonary Medicine Rabin Medical Center Petah Tikva Israel

University Hospital of Pulmonology Clinical Center of Serbia Belgrade Serbia

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