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

Kód výsledku v IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064190%3A_____%2F19%3AN0000079" target="_blank" >RIV/00064190:_____/19:N0000079 - isvavai.cz</a>

Nalezeny alternativní kódy

RIV/00216224:14110/19:00109452 RIV/00216208:11110/19:10394213 RIV/00216208:11130/19:10394213 RIV/00216208:11140/19:10394213 a 8 dalších

Výsledek na webu

<a href="http://dx.doi.org/10.1186/s12931-019-0977-2" target="_blank" >http://dx.doi.org/10.1186/s12931-019-0977-2</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1186/s12931-019-0977-2" target="_blank" >10.1186/s12931-019-0977-2</a>

Jazyk výsledku

angličtina

Název v původním jazyce

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

Popis výsledku v původním jazyce

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.

Název v anglickém jazyce

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

Popis výsledku anglicky

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.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30203 - Respiratory systems

Projekt

—

Návaznosti

V - Vyzkumna aktivita podporovana z jinych verejnych zdroju

Rok uplatnění

2019

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

RESPIRATORY RESEARCH

ISSN

1465-993X

e-ISSN

1465-9921

Svazek periodika

20

Číslo periodika v rámci svazku

01.2019

Stát vydavatele periodika

GB - Spojené království Velké Británie a Severního Irska

Počet stran výsledku

11

Strana od-do

Article Number: 16

Kód UT WoS článku

000458078000002

EID výsledku v databázi Scopus

2-s2.0-85060202895