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

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dc.contributor.authorTran, Tanja
dc.contributor.authorSterclova, Martina
dc.contributor.authorMogulkoc, Nesrin
dc.contributor.authorLewandowska, Katarzyna
dc.contributor.authorMuller, Veronika
dc.contributor.authorHajkova, Marta
dc.contributor.authorMartusewia-Boros, Magdalena
dc.date.accessioned2020-12-01T12:04:57Z
dc.date.available2020-12-01T12:04:57Z
dc.date.issued2020
dc.departmentEge Üniversitesien_US
dc.description.abstractBackground 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.en_US
dc.description.sponsorshipBoehringer IngelheimBoehringer Ingelheimen_US
dc.description.sponsorshipThe EMPIRE registry is supported by a grant from Boehringer Ingelheim (since 2014). the sponsor did not have any involvement in the collection, analysis, and interpretation of the data or in the writing of or commenting on this manuscripten_US
dc.identifier.doi10.1186/s12931-019-1271-zen_US
dc.identifier.issn1465-993X
dc.identifier.issue1en_US
dc.identifier.pmid31915023en_US
dc.identifier.scopus2-s2.0-85077714238en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.urihttps://doi.org/10.1186/s12931-019-1271-z
dc.identifier.urihttps://hdl.handle.net/11454/62826
dc.identifier.volume21en_US
dc.identifier.wosWOS:000512718200003en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherBmcen_US
dc.relation.ispartofRespiratory Researchen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.titleThe European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosisen_US
dc.typeArticleen_US

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