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@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 and 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, vol.~17, No~9, p.~1-13. ISSN~1932-6203. Available from: https://dx.doi.org/10.1371/journal.pone.0273854.
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