Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry
| Autor: | Monika Zurkova, Eva Kriegova, Vitezslav Kolek, Vladimira Lostakova, Martina Sterclova, Vladimir Bartos, Martina Doubkova, Ilona Binkova, Michal Svoboda, Jana Strenkova, 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, Martina Vasakova, ILD section, IPF registry |
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| Jazyk: | angličtina |
| Rok vydání: | 2019 |
| Předmět: |
Male
Vital capacity medicine.medical_specialty Pyridones Vital Capacity Idiopathic pulmonary fibrosis Pirfenidone Cohort Studies 03 medical and health sciences chemistry.chemical_compound FEV1/FVC ratio 0302 clinical medicine DLCO Internal medicine Medicine Humans Lung volumes 030212 general & internal medicine Registries Aged Czech Republic lcsh:RC705-779 Disease progression business.industry Research Anti-Inflammatory Agents Non-Steroidal lcsh:Diseases of the respiratory system respiratory system Middle Aged medicine.disease 3. Good health respiratory tract diseases Respiratory Function Tests Survival Rate Treatment Outcome 030228 respiratory system chemistry Mortality prediction Cohort Nintedanib Female business medicine.drug Follow-Up Studies |
| Zdroj: | Respiratory Research Respiratory Research, Vol 20, Iss 1, Pp 1-11 (2019) |
| ISSN: | 1465-993X 1465-9921 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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