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

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...

Teljes leírás

Elmentve itt :
Bibliográfiai részletek
Szerzők: Tran Tanja
Šterclová Martina
Mogulkoc Nesrin
Lewandowska Katarzyna
Müller Veronika
Hájková Marta
Kramer Mordechai R.
Jovanović Dragana
Kollaborációs szervezet: EMPIRE registry
Zsiray Miklós
Bohács Anikó
Balikó Zoltán
Lajkó Imre
Szalai Zsuzsanna
Petz Aladár
Szilasi Mária
Dokumentumtípus: Cikk
Megjelent: 2020
Sorozat:RESPIRATORY RESEARCH 21 No. 1
Tárgyszavak:
doi:10.1186/s12931-019-1271-z

mtmt:31128601
Online Access:http://publicatio.bibl.u-szeged.hu/29977
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520 3 |a 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.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.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).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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700 0 1 |a Šterclová Martina  |e aut 
700 0 1 |a Mogulkoc Nesrin  |e aut 
700 0 1 |a Lewandowska Katarzyna  |e aut 
700 0 1 |a Müller Veronika  |e aut 
700 0 1 |a Hájková Marta  |e aut 
700 0 1 |a Kramer Mordechai R.  |e aut 
700 0 1 |a Jovanović Dragana  |e aut 
700 0 2 |a Kollaborációs szervezet: EMPIRE registry  |e aut 
700 0 2 |a Zsiray Miklós  |e aut 
700 0 2 |a Bohács Anikó  |e aut 
700 0 2 |a Balikó Zoltán  |e aut 
700 0 2 |a Lajkó Imre  |e aut 
700 0 2 |a Szalai Zsuzsanna  |e aut 
700 0 2 |a Petz Aladár  |e aut 
700 0 2 |a Szilasi Mária  |e aut 
856 4 0 |u http://publicatio.bibl.u-szeged.hu/29977/1/s12931-019-1271-z.pdf  |z Dokumentum-elérés