Background: Pirfenidone slows down disease progression in idiopathic pulmonary fibrosis (IPF). Recent studies suggest a treatment effect in progressive pulmonary fibrosis other than IPF. However, the safety and effectiveness of pirfenidone in asbestosis patients remain unclear. In this study, we aimed to investigate the safety, tolerability and efficacy of pirfenidone in asbestosis patients with a progressive phenotype. Methods: This was a multicenter prospective study in asbestosis patients with progressive lung function decline. After a 12-week observational period, patients were treated with pirfenidone 801 mg three times a day. Symptoms and adverse events were evaluated weekly and patients completed online patient-reported outcomes measures. At baseline, start of therapy, 12 and 24 weeks, in hospital measurement of lung function and a 6 min walking test were performed. Additionally, patients performed daily home spirometry measurements. Results: In total, 10 patients were included of whom 6 patients (66.7%) experienced any adverse events during the study period. Most frequently reported adverse events were fatigue, rash, anorexia and cough, which mostly occurred intermittently and were reported as not very bothersome. No significant changes in hospital pulmonary function (forced vital capacity (FVC), diffusion capacity of the lung for carbon monoxide (DLCO), 6 min walking test or patient-reported outcomes measures before and after start of pirfenidone were found. Home spirometry demonstrated a FVC decline in 12 weeks before start of pirfenidone, while FVC did not decline during the 24 week treatment phase, but this difference was not statistically significant. Conclusions: Treatment with pirfenidone in asbestosis has an acceptable safety and tolerability profile and home spirometry data suggest this antifibrotic treatment might attenuate FVC decline in progressive asbestosis.
|Early online date||28 May 2022|
|Publication status||Published - Dec 2022|
Bibliographical noteFunding Information:
JM reports consulting fees from Boehringer Ingelheim; lecture fees from Boehringer Ingelheim, F. Hoffmann-La Roche, Novartis, outside the submitted work. CM reports grants and fees from Boehringer-Ingelheim and Hoffmann-la Roche, outside the submitted work. All grants and fees were paid to the institution. MV reports consulting fees and lecture honoraria from Boehringer Ingelheim; lecture honoraria from Chiesi; outside the submitted work. MW reports grants from Boehringer Ingelheim, F. Hoffmann-La Roche consulting fees from Boehringer Ingelheim, F. Hoffmann-La Roche, Galapagos, Bristol Myers Squibb, Galecto and Respivant; lecture fees from Boehringer Ingelheim, F. Hoffmann-La Roche, Novartis; travel support from Boehringer Ingelheim, F. Hoffmann-La Roche; and participation in data safety monitoring boards for Savara and Galapagos. All grants and fees were paid to the institution. RM reports consulting fees and lecture honoraria from Boehringer Ingelheim; consulting honoraria from Galapagos; outside the submitted work. All other authors declare that they have no competing interests.
This study by the Dutch Association of Pulmonologists (NVALT) was financially supported by Roche. Roche had no role in the design, analysis and interpretation of the results in this study, but was given the opportunity to review the manuscript for accuracy and intellectual property considerations as it relates to a Roche product.
© 2022, The Author(s).