Effect of pirfenidone on lung function decline and survival:
5-yr experience from a real-life IPF cohort from the Czech
EMPIRE registry

J 2019

Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry

ZURKOVA, Monika; Eva KRIEGOVA; Vitezslav KOLEK; Vladimira LOSTAKOVA; Martina STERCLOVA et. al.

Basic information

Original name

Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry

Authors

ZURKOVA, Monika (203 Czech Republic); Eva KRIEGOVA (203 Czech Republic, guarantor); Vitezslav KOLEK (203 Czech Republic); Vladimira LOSTAKOVA (203 Czech Republic); Martina STERCLOVA (203 Czech Republic); Vladimir BARTOS (203 Czech Republic); Martina DOUBKOVÁ (203 Czech Republic, belonging to the institution); Ilona BINKOVÁ (203 Czech Republic); Michal SVOBODA (203 Czech Republic, belonging to the institution); Jana STRENKOVÁ (203 Czech Republic, belonging to the institution); Marketa JANOTOVA (203 Czech Republic); Martina PLACKOVA (203 Czech Republic); Ladislav LACINA (203 Czech Republic); Vladimir RIHAK (203 Czech Republic); Frantisek PETRIK (203 Czech Republic); Pavlina LISA (203 Czech Republic); Radka BITTENGLOVA (203 Czech Republic); Richard TYL (203 Czech Republic); Gustav ONDREJKA (203 Czech Republic); Hana SULDOVA (203 Czech Republic); Jaroslav LNENICKA (203 Czech Republic); Jana PSIKALOVA (203 Czech Republic); Tomas SNIZEK (203 Czech Republic); Jiri HOMOLKA (203 Czech Republic); Renata KRALOVA (203 Czech Republic); Jan KERVITZER (203 Czech Republic) and Martina VASAKOVA (203 Czech Republic)

Edition

RESPIRATORY RESEARCH, LONDON, BMC, 2019, 1465-993X

Other information

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

References:

URL

RIV identification code

RIV/00216224:14110/19:00109452

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.1186/s12931-019-0977-2

UT WoS

000458078000002

EID Scopus

2-s2.0-85060202895

Keywords in English

Idiopathic pulmonary fibrosis; Pirfenidone; Mortality prediction; Disease progression

Abstract

In the original language

Pirfenidone, an antifibrotic drug, slows-down the disease progression in idiopathic pulmonary fibrosis (IPF) over 12 months, however limited data on the decline of lung function and overall survival (OS) in real-world cohorts on longer follow-up exists. Of the enrolled Czech IPF patients (n = 841) from an EMPIRE registry, 383 (45.5%) received pirfenidone, 218 (25.9%) no-antifibrotic treatment and 240 (28.5%) were excluded (missing data, nintedanib treatment). The 2- and 5-yrs OS and forced vital capacity (FVC) and diffusing lung capacity for carbon monoxide (DLCO) were investigated at treatment initiation and 6, 12, 18 and 24 months' follow-up. During a 2-yr follow-up, less than a quarter of the patients progressed on pirfenidone as assessed by the decline of ae10% FVC (17.0%) and ae 15% DLCO (14.3%). On pirfenidone, the DLCO (ae10%) declines at 6, 12, 18 and 24 months' and DLCO (ae15%) declines at 6, 18 and 24 months' follow-up were associated with increased mortality. The DLCO decline showed higher predictive value for mortality than FVC decline. In patients with no-antifibrotics, FVC and DLCO declines were not predictive for mortality. Pirfenidone increased 5-yrs OS over no-antifibrotic treatment (55.9% vs 31.5% alive, P = 0.002). Our study observed the 2-yrs sustained effect of pirfenidone on the decline of lung function and survival in the real-world patient's IPF cohort. DLCO decline of ae10% shows a potential as a mortality predictor in IPF patients on pirfenidone, and should be routinely evaluated during follow-up examinations.

Cite

ZURKOVA, Monika; Eva KRIEGOVA; Vitezslav KOLEK; Vladimira LOSTAKOVA; Martina STERCLOVA; Vladimir BARTOS; Martina DOUBKOVÁ; Ilona BINKOVÁ; Michal SVOBODA; Jana STRENKOVÁ; Marketa JANOTOVA; Martina PLACKOVA; Ladislav LACINA; Vladimir RIHAK; Frantisek PETRIK; Pavlina LISA; Radka BITTENGLOVA; Richard TYL; Gustav ONDREJKA; Hana SULDOVA; Jaroslav LNENICKA; Jana PSIKALOVA; Tomas SNIZEK; Jiri HOMOLKA; Renata KRALOVA; Jan KERVITZER and Martina VASAKOVA. Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry. RESPIRATORY RESEARCH. LONDON: BMC, 2019, vol. 20, No 16, p. 1-11. ISSN 1465-993X. Available from: https://dx.doi.org/10.1186/s12931-019-0977-2.

@article{1521076,
   author = {Zurkova, Monika and Kriegova, Eva and Kolek, Vitezslav and Lostakova, Vladimira and Sterclova, Martina and Bartos, Vladimir and Doubková, Martina and Binková, Ilona and Svoboda, Michal and Strenková, Jana and Janotova, Marketa and Plackova, Martina and Lacina, Ladislav and Rihak, Vladimir and Petrik, Frantisek and Lisa, Pavlina and Bittenglova, Radka and Tyl, Richard and Ondrejka, Gustav and Suldova, Hana and Lnenicka, Jaroslav and Psikalova, Jana and Snizek, Tomas and Homolka, Jiri and Kralova, Renata and Kervitzer, Jan and Vasakova, Martina},
   article_location = {LONDON},
   article_number = {16},
   doi = {http://dx.doi.org/10.1186/s12931-019-0977-2},
   keywords = {Idiopathic pulmonary fibrosis; Pirfenidone; Mortality prediction; Disease progression},
   language = {eng},
   issn = {1465-993X},
   journal = {RESPIRATORY RESEARCH},
   title = {Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry},
   url = {http://dx.doi.org/10.1186/s12931-019-0977-2},
   volume = {20},
   year = {2019}
}

TY  - JOUR
ID  - 1521076
AU  - Zurkova, Monika - Kriegova, Eva - Kolek, Vitezslav - Lostakova, Vladimira - Sterclova, Martina - Bartos, Vladimir - Doubková, Martina - Binková, Ilona - Svoboda, Michal - Strenková, Jana - Janotova, Marketa - Plackova, Martina - Lacina, Ladislav - Rihak, Vladimir - Petrik, Frantisek - Lisa, Pavlina - Bittenglova, Radka - Tyl, Richard - Ondrejka, Gustav - Suldova, Hana - Lnenicka, Jaroslav - Psikalova, Jana - Snizek, Tomas - Homolka, Jiri - Kralova, Renata - Kervitzer, Jan - Vasakova, Martina
PY  - 2019
TI  - Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry
JF  - RESPIRATORY RESEARCH
VL  - 20
IS  - 16
SP  - 1-11
EP  - 1-11
PB  - BMC
SN  - 1465993X
KW  - Idiopathic pulmonary fibrosis
KW  - Pirfenidone
KW  - Mortality prediction
KW  - Disease progression
UR  - http://dx.doi.org/10.1186/s12931-019-0977-2
L2  - http://dx.doi.org/10.1186/s12931-019-0977-2
N2  - Pirfenidone, an antifibrotic drug, slows-down the disease progression in idiopathic pulmonary fibrosis (IPF) over 12 months, however limited data on the decline of lung function and overall survival (OS) in real-world cohorts on longer follow-up exists. Of the enrolled Czech IPF patients (n = 841) from an EMPIRE registry, 383 (45.5%) received pirfenidone, 218 (25.9%) no-antifibrotic treatment and 240 (28.5%) were excluded (missing data, nintedanib treatment). The 2- and 5-yrs OS and forced vital capacity (FVC) and diffusing lung capacity for carbon monoxide (DLCO) were investigated at treatment initiation and 6, 12, 18 and 24 months' follow-up. During a 2-yr follow-up, less than a quarter of the patients progressed on pirfenidone as assessed by the decline of ae10% FVC (17.0%) and ae 15% DLCO (14.3%). On pirfenidone, the DLCO (ae10%) declines at 6, 12, 18 and 24 months' and DLCO (ae15%) declines at 6, 18 and 24 months' follow-up were associated with increased mortality. The DLCO decline showed higher predictive value for mortality than FVC decline. In patients with no-antifibrotics, FVC and DLCO declines were not predictive for mortality. Pirfenidone increased 5-yrs OS over no-antifibrotic treatment (55.9% vs 31.5% alive, P = 0.002). Our study observed the 2-yrs sustained effect of pirfenidone on the decline of lung function and survival in the real-world patient's IPF cohort. DLCO decline of ae10% shows a potential as a mortality predictor in IPF patients on pirfenidone, and should be routinely evaluated during follow-up examinations.
ER  -

ZURKOVA, Monika; Eva KRIEGOVA; Vitezslav KOLEK; Vladimira LOSTAKOVA; Martina STERCLOVA; Vladimir BARTOS; Martina DOUBKOVÁ; Ilona BINKOVÁ; Michal SVOBODA; Jana STRENKOVÁ; Marketa JANOTOVA; Martina PLACKOVA; Ladislav LACINA; Vladimir RIHAK; Frantisek PETRIK; Pavlina LISA; Radka BITTENGLOVA; Richard TYL; Gustav ONDREJKA; Hana SULDOVA; Jaroslav LNENICKA; Jana PSIKALOVA; Tomas SNIZEK; Jiri HOMOLKA; Renata KRALOVA; Jan KERVITZER and Martina VASAKOVA. Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry. \textit{RESPIRATORY RESEARCH}. LONDON: BMC, 2019, vol.~20, No~16, p.~1-11. ISSN~1465-993X. Available from: https://dx.doi.org/10.1186/s12931-019-0977-2.

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