Survival and lung function decline in patients with definite,
probable and possible idiopathic pulmonary fibrosis treated
with pirfenidone

J 2022

Survival and lung function decline in patients with definite, probable and possible idiopathic pulmonary fibrosis treated with pirfenidone

MÁJEK, Ondřej, Jakub GREGOR, Nesrin MOGULKOĆ, Katarzyna LEWANDOWSKA, Martina ŠTERCLOVÁ et. al.

Základní údaje

Originální název

Survival and lung function decline in patients with definite, probable and possible idiopathic pulmonary fibrosis treated with pirfenidone

Autoři

MÁJEK, Ondřej (203 Česká republika, domácí), Jakub GREGOR (203 Česká republika, domácí), Nesrin MOGULKOĆ, Katarzyna LEWANDOWSKA, Martina ŠTERCLOVÁ, Veronika MÜLLER, Marta HÁJKOVÁ, Mordechai R KRAMER, Jasna TEKAVEC-TRKANJEC, Dragana JOVANOVIĆ, Michael STUDNICKA, Natalia STOEVA, Kirchgässler KLAUS-UWE, Simona LITTNEROVÁ (203 Česká republika, domácí), Ladislav DUŠEK (203 Česká republika, domácí) a Martina VAŠÁKOVÁ KOZIAR (garant)

Vydání

PLOS One, SAN FRANCISCO, PUBLIC LIBRARY SCIENCE, 2022, 1932-6203

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30203 Respiratory systems

Stát vydavatele

Spojené státy

Utajení

není předmětem státního či obchodního tajemství

Odkazy

URL

Impakt faktor

Impact factor: 3.700

Kód RIV

RIV/00216224:14110/22:00126652

Organizační jednotka

Lékařská fakulta

DOI

http://dx.doi.org/10.1371/journal.pone.0273854

UT WoS

000892263300073

Klíčová slova anglicky

idiopathic pulmonary fibrosis; pirfenidone; survival ; lung function decline

Štítky

14119612, rivok

Příznaky

Mezinárodní význam, Recenzováno

Změněno: 16. 1. 2023 14:05, Mgr. Tereza Miškechová

Anotace

V originále

Background There is no clear evidence whether pirfenidone has a benefit in patients with probable or possible UIP, i.e. when idiopathic pulmonary fibrosis (IPF) is diagnosed with a lower degree of diagnostic certainty. We report on outcomes of treatment with pirfenidone in IPF patients diagnosed with various degrees of certainty. Methods and findings We followed patients in the multi-national European MultiPartner IPF Registry (EMPIRE) first seen between 2015 and 2018. Patients were assessed with HRCT, histopathology and received a multi-disciplinary team (MDT) IPF diagnosis. Endpoints of interest were overall survival (OS), progression-free survival (PFS) and lung function decline. Results A total of 1626 patients were analysed, treated with either pirfenidone (N = 808) or receiving no antifibrotic treatment (N = 818). When patients treated with pirfenidone were compared to patients not receiving antifibrotic treatment, OS (one-, two- and three-year probability of survival 0.871 vs 0.798; 0.728 vs 0.632; 0.579 vs 0.556, P = 0.002), and PFS (one-, two- and three-year probability of survival 0.597 vs 0.536; 0.309 vs 0.281; 0.158 vs 0.148, P = 0.043) was higher, and FVC decline smaller (-0.073 l/yr vs -0.169 l/yr, P = 0.017). The benefit of pirfenidone on OS and PFS was also seen in patients with probable or possible IPF. Conclusions This EMPIRE analysis confirms the favourable outcomes observed for pirfenidone treatment in patients with definitive IPF and indicates benefits also for patients with probable or possible IPF.

Citovat

MÁJEK, Ondřej, Jakub GREGOR, Nesrin MOGULKOĆ, Katarzyna LEWANDOWSKA, Martina ŠTERCLOVÁ, Veronika MÜLLER, Marta HÁJKOVÁ, Mordechai R KRAMER, Jasna TEKAVEC-TRKANJEC, Dragana JOVANOVIĆ, Michael STUDNICKA, Natalia STOEVA, Kirchgässler KLAUS-UWE, Simona LITTNEROVÁ, Ladislav DUŠEK a Martina VAŠÁKOVÁ KOZIAR. Survival and lung function decline in patients with definite, probable and possible idiopathic pulmonary fibrosis treated with pirfenidone. PLOS One. SAN FRANCISCO: PUBLIC LIBRARY SCIENCE, 2022, roč. 17, č. 9, s. 1-13. ISSN 1932-6203. Dostupné z: https://dx.doi.org/10.1371/journal.pone.0273854.

@article{2217372,
   author = {Májek, Ondřej and Gregor, Jakub and Mogulkoć, Nesrin and Lewandowska, Katarzyna and Šterclová, Martina and Müller, Veronika and Hájková, Marta and Kramer, Mordechai R and TekavecandTrkanjec, Jasna and Jovanović, Dragana and Studnicka, Michael and Stoeva, Natalia and KlausandUwe, Kirchgässler and Littnerová, Simona and Dušek, Ladislav and Vašáková Koziar, Martina},
   article_location = {SAN FRANCISCO},
   article_number = {9},
   doi = {http://dx.doi.org/10.1371/journal.pone.0273854},
   keywords = {idiopathic pulmonary fibrosis; pirfenidone; survival ; lung function decline},
   language = {eng},
   issn = {1932-6203},
   journal = {PLOS One},
   title = {Survival and lung function decline in patients with definite, probable and possible idiopathic pulmonary fibrosis treated with pirfenidone},
   url = {https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0273854},
   volume = {17},
   year = {2022}
}

TY  - JOUR
ID  - 2217372
AU  - Májek, Ondřej - Gregor, Jakub - Mogulkoć, Nesrin - Lewandowska, Katarzyna - Šterclová, Martina - Müller, Veronika - Hájková, Marta - Kramer, Mordechai R - Tekavec-Trkanjec, Jasna - Jovanović, Dragana - Studnicka, Michael - Stoeva, Natalia - Klaus-Uwe, Kirchgässler - Littnerová, Simona - Dušek, Ladislav - Vašáková Koziar, Martina
PY  - 2022
TI  - Survival and lung function decline in patients with definite, probable and possible idiopathic pulmonary fibrosis treated with pirfenidone
JF  - PLOS One
VL  - 17
IS  - 9
SP  - 1-13
EP  - 1-13
PB  - PUBLIC LIBRARY SCIENCE
SN  - 19326203
KW  - idiopathic pulmonary fibrosis
KW  - pirfenidone
KW  - survival
KW  - lung function decline
UR  - https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0273854
N2  - Background There is no clear evidence whether pirfenidone has a benefit in patients with probable or possible UIP, i.e. when idiopathic pulmonary fibrosis (IPF) is diagnosed with a lower degree of diagnostic certainty. We report on outcomes of treatment with pirfenidone in IPF patients diagnosed with various degrees of certainty. Methods and findings We followed patients in the multi-national European MultiPartner IPF Registry (EMPIRE) first seen between 2015 and 2018. Patients were assessed with HRCT, histopathology and received a multi-disciplinary team (MDT) IPF diagnosis. Endpoints of interest were overall survival (OS), progression-free survival (PFS) and lung function decline. Results A total of 1626 patients were analysed, treated with either pirfenidone (N = 808) or receiving no antifibrotic treatment (N = 818). When patients treated with pirfenidone were compared to patients not receiving antifibrotic treatment, OS (one-, two- and three-year probability of survival 0.871 vs 0.798; 0.728 vs 0.632; 0.579 vs 0.556, P = 0.002), and PFS (one-, two- and three-year probability of survival 0.597 vs 0.536; 0.309 vs 0.281; 0.158 vs 0.148, P = 0.043) was higher, and FVC decline smaller (-0.073 l/yr vs -0.169 l/yr, P = 0.017). The benefit of pirfenidone on OS and PFS was also seen in patients with probable or possible IPF. Conclusions This EMPIRE analysis confirms the favourable outcomes observed for pirfenidone treatment in patients with definitive IPF and indicates benefits also for patients with probable or possible IPF.
ER  -

MÁJEK, Ondřej, Jakub GREGOR, Nesrin MOGULKO$\backslash$'C, Katarzyna LEWANDOWSKA, Martina ŠTERCLOVÁ, Veronika MÜLLER, Marta HÁJKOVÁ, Mordechai R KRAMER, Jasna TEKAVEC-TRKANJEC, Dragana JOVANOVI$\backslash$'C, Michael STUDNICKA, Natalia STOEVA, Kirchgässler KLAUS-UWE, Simona LITTNEROVÁ, Ladislav DUŠEK a Martina VAŠÁKOVÁ KOZIAR. Survival and lung function decline in patients with definite, probable and possible idiopathic pulmonary fibrosis treated with pirfenidone. \textit{PLOS One}. SAN FRANCISCO: PUBLIC LIBRARY SCIENCE, 2022, roč.~17, č.~9, s.~1-13. ISSN~1932-6203. Dostupné z: https://dx.doi.org/10.1371/journal.pone.0273854.

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