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Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry
M. Zurkova, E. Kriegova, V. Kolek, V. Lostakova, M. Sterclova, V. Bartos, M. Doubkova, I. Binkova, M. Svoboda, J. Strenkova, M. Janotova, M. Plackova, L. Lacina, V. Rihak, F. Petrik, P. Lisa, R. Bittenglova, R. Tyl, G. Ondrejka, H. Suldova, J....
Language English Country England, Great Britain
Document type Journal Article
NLK
BioMedCentral
from 2000-01-04
BioMedCentral Open Access
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Directory of Open Access Journals
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Free Medical Journals
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PubMed Central
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Europe PubMed Central
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- MeSH
- Anti-Inflammatory Agents, Non-Steroidal pharmacology therapeutic use MeSH
- Idiopathic Pulmonary Fibrosis diagnosis drug therapy epidemiology MeSH
- Cohort Studies MeSH
- Middle Aged MeSH
- Humans MeSH
- Survival Rate trends MeSH
- Follow-Up Studies MeSH
- Disease Progression * MeSH
- Pyridones pharmacology therapeutic use MeSH
- Registries * MeSH
- Respiratory Function Tests trends MeSH
- Aged MeSH
- Vital Capacity drug effects physiology MeSH
- Treatment Outcome MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Geographicals
- Czech Republic MeSH
INTRODUCTION: 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. PATIENTS/METHODS: 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. RESULTS: During a 2-yr follow-up, less than a quarter of the patients progressed on pirfenidone as assessed by the decline of ≥10% FVC (17.0%) and ≥ 15% DLCO (14.3%). On pirfenidone, the DLCO (≥10%) declines at 6, 12, 18 and 24 months' and DLCO (≥15%) 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). CONCLUSION: 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 ≥10% shows a potential as a mortality predictor in IPF patients on pirfenidone, and should be routinely evaluated during follow-up examinations.
Department of Pulmonary Medicine and Allergology Hospital Kromeriz Kromeriz Czech Republic
Department of Pulmonary Medicine and Thoracic Surgery Hospital Na Bulovce Prague Czech Republic
Department of Pulmonary Medicine Hospital Ceske Budejovice Ceske Budejovice Czech Republic
Department of Pulmonary Medicine Hospital Jihlava Jihlava Czech Republic
Department of Pulmonary Medicine Hospital Novy Jicin Novy Jicin Czech Republic
Department of Pulmonary Medicine Hospital Znojmo Znojmo Czech Republic
Department of Pulmonary Medicine Regional Hospital Pardubice Pardubice Czech Republic
Department of Pulmonary Medicine Tomas Bata Regional Hospital Zlin Czech Republic
Department of Pulmonary Medicine University Hospital Plzen Pilsen Czech Republic
Institute of Biostatistics and Analyses Faculty of Medicine Masaryk University Brno Czech Republic
References provided by Crossref.org
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- $a Zurkova, Monika $u Department of Pulmonary Diseases and Tuberculosis, Faculty of Medicine and Dentistry, Palacky University in Olomouc, University Hospital Olomouc, Olomouc, Czech Republic.
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- $a Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry / $c M. Zurkova, E. Kriegova, V. Kolek, V. Lostakova, M. Sterclova, V. Bartos, M. Doubkova, I. Binkova, M. Svoboda, J. Strenkova, M. Janotova, M. Plackova, L. Lacina, V. Rihak, F. Petrik, P. Lisa, R. Bittenglova, R. Tyl, G. Ondrejka, H. Suldova, J. Lnenicka, J. Psikalova, T. Snizek, J. Homolka, R. Kralova, J. Kervitzer, M. Vasakova, . , . ,
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- $a INTRODUCTION: 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. PATIENTS/METHODS: 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. RESULTS: During a 2-yr follow-up, less than a quarter of the patients progressed on pirfenidone as assessed by the decline of ≥10% FVC (17.0%) and ≥ 15% DLCO (14.3%). On pirfenidone, the DLCO (≥10%) declines at 6, 12, 18 and 24 months' and DLCO (≥15%) 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). CONCLUSION: 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 ≥10% shows a potential as a mortality predictor in IPF patients on pirfenidone, and should be routinely evaluated during follow-up examinations.
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