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.

Základní údaje

Originální název

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

Autoři

ZURKOVA, Monika (203 Česká republika), Eva KRIEGOVA (203 Česká republika, garant), Vitezslav KOLEK (203 Česká republika), Vladimira LOSTAKOVA (203 Česká republika), Martina STERCLOVA (203 Česká republika), Vladimir BARTOS (203 Česká republika), Martina DOUBKOVÁ (203 Česká republika, domácí), Ilona BINKOVÁ (203 Česká republika), Michal SVOBODA (203 Česká republika, domácí), Jana STRENKOVÁ (203 Česká republika, domácí), Marketa JANOTOVA (203 Česká republika), Martina PLACKOVA (203 Česká republika), Ladislav LACINA (203 Česká republika), Vladimir RIHAK (203 Česká republika), Frantisek PETRIK (203 Česká republika), Pavlina LISA (203 Česká republika), Radka BITTENGLOVA (203 Česká republika), Richard TYL (203 Česká republika), Gustav ONDREJKA (203 Česká republika), Hana SULDOVA (203 Česká republika), Jaroslav LNENICKA (203 Česká republika), Jana PSIKALOVA (203 Česká republika), Tomas SNIZEK (203 Česká republika), Jiri HOMOLKA (203 Česká republika), Renata KRALOVA (203 Česká republika), Jan KERVITZER (203 Česká republika) a Martina VASAKOVA (203 Česká republika)

Vydání

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

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30203 Respiratory systems

Stát vydavatele

Velká Británie a Severní Irsko

Utajení

není předmětem státního či obchodního tajemství

Odkazy

URL

Impakt faktor

Impact factor: 3.924

Kód RIV

RIV/00216224:14110/19:00109452

Organizační jednotka

Lékařská fakulta

DOI

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

UT WoS

000458078000002

Klíčová slova anglicky

Idiopathic pulmonary fibrosis; Pirfenidone; Mortality prediction; Disease progression

Štítky

14110215, 14119612, rivok

Příznaky

Mezinárodní význam, Recenzováno

Změněno: 2. 6. 2020 13:56, Mgr. Tereza Miškechová

Anotace

V originále

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.

Citovat

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 a 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, roč. 20, č. 16, s. 1-11. ISSN 1465-993X. Dostupné z: 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 a 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, roč.~20, č.~16, s.~1-11. ISSN~1465-993X. Dostupné z: https://dx.doi.org/10.1186/s12931-019-0977-2.

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