TY  - JOUR
AU  - Tran, Tanja
AU  - Šterclová, Martina
AU  - Mogulkoc, Nesrin
AU  - Lewandowska, Katarzyna
AU  - Müller, Veronika
AU  - Hájková, Marta
AU  - Kramer, Mordechai R
AU  - Jovanović, Dragana
AU  - Tekavec-Trkanjec, Jasna
AU  - Studnicka, Michael
AU  - Stoeva, Natalia
AU  - Hejduk, Karel
AU  - Dušek, Ladislav
AU  - Suissa, Samy
AU  - Vašáková, Martina
ED  - Zolnowska, Beata / Collaborator
ED  - Bartoš, Vladimír / Collaborator
ED  - Králové, Hradec / Collaborator
ED  - Slivka, Robert / Collaborator
ED  - Lacina, Ladislav / Collaborator
ED  - Doubková, Martina / Collaborator
ED  - Bittenglová, Radka / Collaborator
ED  - Martusewicz-Boros, Magdalena / Collaborator
ED  - Žurková, Monika / Collaborator
ED  - Jonner, Imrich / Collaborator
ED  - Szymanowska-Narloch, Amelia / Collaborator
ED  - Sobiecka, Małgorzata / Collaborator
ED  - Lošťáková, Vladimíra / Collaborator
ED  - Trzaska-Sobczak, Marzena / Collaborator
ED  - Tyl, Richard / Collaborator
ED  - Sliwinski, Pawel / Collaborator
ED  - Zsiray, Miklós / Collaborator
ED  - Bohács, Anikó / Collaborator
ED  - Majewski, Sebastian / Collaborator
ED  - Šuldová, Hana / Collaborator
ED  - Lewandowska, Katarzyna / Collaborator
ED  - Matula, Bohumil / Collaborator
ED  - Šišková, Lenka / Collaborator
ED  - Plutinský, Ján / Collaborator
ED  - Jakić, Ana / Collaborator
ED  - Tóth, Štefan / Collaborator
ED  - Balikó, Zoltán / Collaborator
ED  - Bučeková, Margita / Collaborator
ED  - Pšíkalová, Jana / Collaborator
ED  - Stachura, Tomasz / Collaborator
ED  - Petřík, František / Collaborator
ED  - Anton, Jan / Collaborator
ED  - Lněnička, Jaroslav / Collaborator
ED  - Milenkovic, Marina Roksandić / Collaborator
ED  - Lajkó, Imre / Collaborator
ED  - Řihák, Vladimír / Collaborator
ED  - Szalai, Zsuzsanna / Collaborator
ED  - Jurek, Paulina / Collaborator
ED  - Kania, Aleksander / Collaborator
ED  - Laššán, Štefan / Collaborator
ED  - Pejcic, Tatjana / Collaborator
ED  - Reiterer, Pavel / Collaborator
ED  - Borucki, Lukasz / Collaborator
ED  - Králová, Renata / Collaborator
ED  - Musilová, Pavlína / Collaborator
ED  - Snížek, Tomáš / Collaborator
ED  - Doležal, Daniel / Collaborator
ED  - Homolka, Jiří / Collaborator
ED  - Hortvíková, Hana / Collaborator
ED  - Mladinov, Suzana / Collaborator
ED  - Palúch, Peter / Collaborator
ED  - Hrdina, Roman / Collaborator
ED  - Szilasi, Mária / Collaborator
ED  - Vučinić-Mihailović, Violeta / Collaborator
ED  - Vyšehradský, Róbert / Collaborator
ED  - Mokošová, Radka / Collaborator
ED  - Nowicka, Agata / Collaborator
ED  - Petkovic, Tatjana Radjenovic / Collaborator
TI  - The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis
JF  - RESPIRATORY RESEARCH
J2  - RESP RES
VL  - 21
PY  - 2020
IS  - 1
PG  - 9
SN  - 1465-9921
DO  - 10.1186/s12931-019-1271-z
UR  - https://m2.mtmt.hu/api/publication/31128601
ID  - 31128601
AB  - 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.
LA  - English
DB  - MTMT
ER  -