Treatment of idiopathic pulmonary fibrosis (IPF) with Esbriet (pirfenidone) increased five-year survival, and also resulted in less decline in lung function at two years, according to a real-life study from the Czech Republic.
The findings also revealed that a specific measure of lung function called diffusing lung capacity for carbon monoxide (%DLCO) — a test of the lungs’ capacity to transfer oxygen from the air sacs into the blood — could predict mortality in IPF patients using Esbriet.
The research, “Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry,” was published in the journal Respiratory Research.
IPF treatment with Esbriet has been associated with benefits in lung function, exercise tolerance, and progression-free survival, including in patients with advanced disease. Such improvements have been shown in real-life studies in various European countries at 12 months of treatment, but long-term data on disease progression are still lacking.
Aiming to address this gap, a team from the Czech Republic analyzed two- and five-year overall survival and lung function decline in this population, comparing long-term treatment with Genentech’s antifibrotic Esbriet therapy to treatment with no-antifibrotic therapies. The team also explored the relationship between lung function decline and mortality.
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Data from 841 IPF patients were collected prospectively from December 2012 to December 2017 from the Czech IPF registry, part of an international database of patients with IPF in central and eastern Europe (the European MultiPartner IPF Registry, EMPIRE).
Among the 601 patients (431 men, 259 of whom older than 70 years) with complete clinical and demographical data, 383 were treated with Esbriet (2,403 mg/day in three doses), and 218 with no-antifibrotic treatment (including corticosteroids, the antioxidant N-acethylcystein, the immunosuppressant azathioprine, or their combinations).
Overall survival, forced vital capacity (FVC) — a standard measure of lung function — and %DLCO were investigated at treatment start and after six, 12, 18, and 24 months of treatment. Mean follow-up duration was 22.8 months with Esbriet, and 32.1 months with other therapies.
Disease progression, as reflected by a 10% or greater decline in FVC, was observed in 5.3%, 10.7%, 16.6%, and 17.0% of patients on Esbriet at six, 12, 18, and 24 months, respectively. This compared to higher values — 18.3, 25.0, 24.2, and 31.0%, respectively — in patients on no-antifibrotic treatment.
FVC benefits with Esbriet also were found with 5% or greater FVC decline as cut-off — 17.3, 25.2, 35.5, and 34.8% with Esbriet, versus 35.0, 44.4, 51.5, and 50.0% with the other therapies.
In turn, using a %DLCO decline of 15% or greater as marker, 6.1, 11.3, 11.5, and 14.3% of patients on Esbriet, and 8.5, 18.5, 12.5, and 25.6% on those on other treatments, experienced IPF progression at six, 12, 18 and 24 months, respectively.
Using a 10% or greater %DLCO decline, Esbriet led to 15.4, 19.7, 22.4, and 26.3% of patients experiencing disease progression, compared to 18.6, 30.8, 25.0, and 33.3% in the other group at the same timepoints.
Researchers found that %DLCO had significant associations with lung function decline and mortality in Esbriet-treated patients (except at the 12- month timepoint, for a %DLCO decline of 15% or greater). Also, the %DLCO decline showed higher predictive value for mortality than FVC decline.
In patients treated with no-antifibrotics, DLCO and FVC declines were overall not predictive for mortality.
The researchers suggested that a %DLCO decline of 10% or greater “shows a potential as a mortality predictor in IPF patients on pirfenidone [Esbriet], and should be routinely evaluated during follow-up examinations.”
The data also showed a trend toward lower percentages of hospitalized patients with Esbriet (31.9%) than with no-antifibrotics (39.4%). No significant differences were found when comparing the percentage of patients with acute exacerbations.
Importantly, Esbriet prolonged overall survival at 12, 14, and 60 months (five years) compared with the other therapies. At five years, most patients on Esbriet (55.9%) were still alive vs. 31.5% of those on no-antifibrotic treatments.
Also, the results revealed that the deaths during follow-up were mostly due to IPF-related acute exacerbation or suspected acute exacerbation — 67.3% in the Esbriet group, and 73.6% in the no-antifibrotics group.
Overall, “our data proved that pirfenidone [Esbriet] has a 2-years lasting effect on the slowing down of lung function decline, as well as on reducing mortality,” the researchers wrote.