Background and importance Idiopathic pulmonary fibrosis (IPF) is a severe pulmonary disease with few therapeutic alternatives and characterised by progressive decline in lung function. Pirfenidone and nintedanib are tyrosine kinase inhibitors that have been approved for clinical treatment in mild to moderate IPF in adults.
Aim and objectives The aim of this study was to review the use of pirfenidone and nintedanib in terms of effectiveness and safety in patients with mild to moderate IPF over 12 months of follow-up, through indicators such as the volume of air that can be exhaled with maximal effort (FVC) and the alveolar–capillary diffusion of carbon monoxide (DLco).
Material and methods A retrospective observational study was conducted in patients receiving treatment with pirfenidone and nintedanib from January 2018 to October 2020. Clinical data were collected from the electronic web based national register of Italian medicines. Measured variables were: age, sex, pathology severeness, forced vital capacity (FVC%), diffusion capacity of the lungs for carbon monoxide (DLco,%), before and after the start of treatment. The main outcome was the variation in FVC from baseline levels, considering it a positive response to the treatment if FVC did not decrease by more than 10%. The presence of adverse reactions was reviewed to assess safety.
Results In our hospital, we enrolled 70 patients receiving pirfenidone (50%) and nintedanib (50%). 81.4% of patients were men, with a mean age of 74±8 years. 57.1% had moderate pathology and 42.9% had mild pathology. 2.8% of patients left by voluntary decision and another 3.1% had progression of the pathology. The initial median FVC was 79.63±15.14 and median DLco was 51.74±15.13. After treatment, we obtained a median FVC of 78.31±15.65 and a median DLco of 48.09±16.38. 83.8% of treated patients had a positive response with a decrease of no more than 10% of FVC, and 22.1% of treated patients had an improvement in FVC >5%. 11.8% of treated patients had an improvement in DLco >5%. 14 patients had adverse reactions. The most frequent was dyspepsia (36%), followed by rash (25%); less frequent side effect were headache (8.4%) and diarrhoea (8.4%).
Conclusion and relevance Pirfenidone and nintedanib showed benefit in decreasing progression of IPF and had effectiveness and safety profiles similar to those of other studies, revealing that it is a well tolerated and effective drug. On the other hand, the adverse reactions were mild, but the rate of occurrence was high.
Conflict of interest No conflict of interest
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.