TRAN, T., M. STERCLOVA, N. MOGULKOC, K. LEWANDOWSKA, V. MULLER, M. HAJKOVA, M. R. KRAMER, D. JOVANOVIC, J. TEKAVEC-TRKANJEC, M. STUDNICKA, N. STOEVA, Karel HEJDUK, Ladislav DUŠEK, S. SUISSA, M. VASAKOVA, B. ZOLNOWSKA, V. BARTOS, M. PLACKOVA, R. SLIVKA, L. LACINA, M. DOUBKOVA, R. BITTENGLOVA, M. MARTUSEWIA-BOROS, M. ZURKOVA, I. JONNER, A. SZYMANOWSKA-NARIOCH, I. BINKOVA, M. SOBIECKA, V. LOSTAKOVA, M. TRZASKA-SOBCZAK, R. TYL, P. SLIWINSKI, M. ZSIRAY, P. LISA, A. BOHACS, S. MAJEWSKI, H. SULDOVA, B. MATULA, L. SISKOVA, J. PLUTINSKY, A. JAKIC, S. TOTH, Z. BALIKO, M. BUCEKOVA, J. PSIKALOVA, T. STACHURA, F. PETRIK, J. ANTON, J. LNENICKA, M. R. MILENKOVIC, I. LAJKO, V. RIHAK, Z. SZALAI, P. JUREK, A. KANIA, S. LASSAN, T. PEJCIC, P. REITERER, L. BORUCKI, R. KRALOVA, P. MUSILOVA, T. SNIZEK, D. DOLEZAL, J. HOMOLKA, H. HORTVIKOVA, S. MLADINOV, P. PALUCH, R. HRDINA, M. SZILASI, V. VUCINIC-MIHAILOVIC, R. VYSEHRADSKY, R. MOKOSOVA, A. NOWICKA and T. R. PETKOVIC. The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis. RESPIRATORY RESEARCH. LONDON: BMC, 2020, vol. 21, No 1, p. 1-9. ISSN 1465-9921. Available from: https://dx.doi.org/10.1186/s12931-019-1271-z.
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Basic information
Original name The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis
Authors TRAN, T. (124 Canada), M. STERCLOVA (203 Czech Republic), N. MOGULKOC (792 Turkey), K. LEWANDOWSKA (616 Poland), V. MULLER (348 Hungary), M. HAJKOVA (703 Slovakia), M. R. KRAMER (376 Israel), D. JOVANOVIC (688 Serbia), J. TEKAVEC-TRKANJEC (191 Croatia), M. STUDNICKA (40 Austria), N. STOEVA (100 Bulgaria), Karel HEJDUK (203 Czech Republic, belonging to the institution), Ladislav DUŠEK (203 Czech Republic, belonging to the institution), S. SUISSA (124 Canada), M. VASAKOVA (203 Czech Republic), B. ZOLNOWSKA (616 Poland), V. BARTOS (203 Czech Republic), M. PLACKOVA (203 Czech Republic), R. SLIVKA (703 Slovakia), L. LACINA (203 Czech Republic), M. DOUBKOVA (203 Czech Republic), R. BITTENGLOVA (203 Czech Republic), M. MARTUSEWIA-BOROS (616 Poland), M. ZURKOVA (203 Czech Republic), I. JONNER (703 Slovakia), A. SZYMANOWSKA-NARIOCH (616 Poland), I. BINKOVA (203 Czech Republic), M. SOBIECKA (616 Poland), V. LOSTAKOVA (203 Czech Republic), M. TRZASKA-SOBCZAK (616 Poland), R. TYL (203 Czech Republic), P. SLIWINSKI (616 Poland), M. ZSIRAY (348 Hungary), P. LISA (203 Czech Republic), A. BOHACS (348 Hungary), S. MAJEWSKI (616 Poland), H. SULDOVA (203 Czech Republic), B. MATULA (616 Poland), L. SISKOVA (703 Slovakia), J. PLUTINSKY (703 Slovakia), A. JAKIC (688 Serbia), S. TOTH (703 Slovakia), Z. BALIKO (348 Hungary), M. BUCEKOVA (703 Slovakia), J. PSIKALOVA (203 Czech Republic), T. STACHURA (616 Poland), F. PETRIK (203 Czech Republic), J. ANTON (203 Czech Republic), J. LNENICKA (203 Czech Republic), M. R. MILENKOVIC (688 Serbia), I. LAJKO (348 Hungary), V. RIHAK (203 Czech Republic), Z. SZALAI (348 Hungary), P. JUREK (616 Poland), A. KANIA (616 Poland), S. LASSAN (703 Slovakia), T. PEJCIC (688 Serbia), P. REITERER (203 Czech Republic), L. BORUCKI (616 Poland), R. KRALOVA (203 Czech Republic), P. MUSILOVA (203 Czech Republic), T. SNIZEK (203 Czech Republic), D. DOLEZAL (203 Czech Republic), J. HOMOLKA (203 Czech Republic), H. HORTVIKOVA (203 Czech Republic), S. MLADINOV (191 Croatia), P. PALUCH (203 Czech Republic), R. HRDINA (203 Czech Republic), M. SZILASI (348 Hungary), V. VUCINIC-MIHAILOVIC (688 Serbia), R. VYSEHRADSKY (703 Slovakia), R. MOKOSOVA (203 Czech Republic), A. NOWICKA (616 Poland) and T. R. PETKOVIC (203 Czech Republic).
Edition RESPIRATORY RESEARCH, LONDON, BMC, 2020, 1465-9921.
Other information
Original language English
Type of outcome Article in a journal
Field of Study 30203 Respiratory systems
Country of publisher United Kingdom of Great Britain and Northern Ireland
Confidentiality degree is not subject to a state or trade secret
WWW URL
Impact factor Impact factor: 2.859 in 2010
RIV identification code RIV/00216224:14110/20:00115999
Organization unit Faculty of Medicine
Doi http://dx.doi.org/10.1186/s12931-019-1271-z
UT WoS 000512718200003
Keywords in English CLINICAL-COURSE; STAGING SYSTEM; GAP SCORE; SURVIVAL; HYPERTENSION; PIRFENIDONE; EFFICACY
Tags 14119612, rivok
Tags International impact, Reviewed
Changed by Changed by: Mgr. Tereza Miškechová, učo 341652. Changed: 12/5/2021 13:13.
Abstract
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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