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

J 2020

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

TRAN, T., M. STERCLOVA, N. MOGULKOC, K. LEWANDOWSKA, V. MULLER et. al.

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

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30203 Respiratory systems

Country of publisher

United Kingdom of Great Britain and Northern Ireland

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

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

Abstract

V originále

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.

Citovat

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.

@article{1670460,
   author = {Tran, T. and Sterclova, M. and Mogulkoc, N. and Lewandowska, K. and Muller, V. and Hajkova, M. and Kramer, M. R. and Jovanovic, D. and TekavecandTrkanjec, J. and Studnicka, M. and Stoeva, N. and Hejduk, Karel and Dušek, Ladislav and Suissa, S. and Vasakova, M. and Zolnowska, B. and Bartos, V. and Plackova, M. and Slivka, R. and Lacina, L. and Doubkova, M. and Bittenglova, R. and MartusewiaandBoros, M. and Zurkova, M. and Jonner, I. and SzymanowskaandNarioch, A. and Binkova, I. and Sobiecka, M. and Lostakova, V. and TrzaskaandSobczak, M. and Tyl, R. and Sliwinski, P. and Zsiray, M. and Lisa, P. and Bohacs, A. and Majewski, S. and Suldova, H. and Matula, B. and Siskova, L. and Plutinsky, J. and Jakic, A. and Toth, S. and Baliko, Z. and Bucekova, M. and Psikalova, J. and Stachura, T. and Petrik, F. and Anton, J. and Lnenicka, J. and Milenkovic, M. R. and Lajko, I. and Rihak, V. and Szalai, Z. and Jurek, P. and Kania, A. and Lassan, S. and Pejcic, T. and Reiterer, P. and Borucki, L. and Kralova, R. and Musilova, P. and Snizek, T. and Dolezal, D. and Homolka, J. and Hortvikova, H. and Mladinov, S. and Paluch, P. and Hrdina, R. and Szilasi, M. and VucinicandMihailovic, V. and Vysehradsky, R. and Mokosova, R. and Nowicka, A. and Petkovic, T. R.},
   article_location = {LONDON},
   article_number = {1},
   doi = {http://dx.doi.org/10.1186/s12931-019-1271-z},
   keywords = {CLINICAL-COURSE; STAGING SYSTEM; GAP SCORE; SURVIVAL; HYPERTENSION; PIRFENIDONE; EFFICACY},
   language = {eng},
   issn = {1465-9921},
   journal = {RESPIRATORY RESEARCH},
   title = {The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis},
   url = {https://respiratory-research.biomedcentral.com/track/pdf/10.1186/s12931-019-1271-z},
   volume = {21},
   year = {2020}
}

TY  - JOUR
ID  - 1670460
AU  - Tran, T. - Sterclova, M. - Mogulkoc, N. - Lewandowska, K. - Muller, V. - Hajkova, M. - Kramer, M. R. - Jovanovic, D. - Tekavec-Trkanjec, J. - Studnicka, M. - Stoeva, N. - Hejduk, Karel - Dušek, Ladislav - Suissa, S. - Vasakova, M. - Zolnowska, B. - Bartos, V. - Plackova, M. - Slivka, R. - Lacina, L. - Doubkova, M. - Bittenglova, R. - Martusewia-Boros, M. - Zurkova, M. - Jonner, I. - Szymanowska-Narioch, A. - Binkova, I. - Sobiecka, M. - Lostakova, V. - Trzaska-Sobczak, M. - Tyl, R. - Sliwinski, P. - Zsiray, M. - Lisa, P. - Bohacs, A. - Majewski, S. - Suldova, H. - Matula, B. - Siskova, L. - Plutinsky, J. - Jakic, A. - Toth, S. - Baliko, Z. - Bucekova, M. - Psikalova, J. - Stachura, T. - Petrik, F. - Anton, J. - Lnenicka, J. - Milenkovic, M. R. - Lajko, I. - Rihak, V. - Szalai, Z. - Jurek, P. - Kania, A. - Lassan, S. - Pejcic, T. - Reiterer, P. - Borucki, L. - Kralova, R. - Musilova, P. - Snizek, T. - Dolezal, D. - Homolka, J. - Hortvikova, H. - Mladinov, S. - Paluch, P. - Hrdina, R. - Szilasi, M. - Vucinic-Mihailovic, V. - Vysehradsky, R. - Mokosova, R. - Nowicka, A. - Petkovic, T. R.
PY  - 2020
TI  - The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis
JF  - RESPIRATORY RESEARCH
VL  - 21
IS  - 1
SP  - 1-9
EP  - 1-9
PB  - BMC
SN  - 14659921
KW  - CLINICAL-COURSE
KW  - STAGING SYSTEM
KW  - GAP SCORE
KW  - SURVIVAL
KW  - HYPERTENSION
KW  - PIRFENIDONE
KW  - EFFICACY
UR  - https://respiratory-research.biomedcentral.com/track/pdf/10.1186/s12931-019-1271-z
L2  - https://respiratory-research.biomedcentral.com/track/pdf/10.1186/s12931-019-1271-z
N2  - 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.
ER  -

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. \textit{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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