Background Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease characterised by chronic, progressive fibrosis, progressive respiratory failure and high mortality. The main goal of treating IPF is to stabilise or reduce the rate of disease progression. Over the past 5 years, two novel antifibrotic therapies, pirfenidone and nintedanib, have been developed, providing treatment options for many patients with IPF.
Purpose To evaluate the efficacy and safety of the antifibrotic treatment with pirfenidone or nintedanib in patients affected by IPF.
Material and methods Retrospective observational study including all patients treated with antifibrotics until September 2018. Using the ATHOS program and their clinical history, we registered: sex, age, previous treatment, start date of antifibrotic, dose reduction, exacerbations experienced, and initial and final forced vital capacity (FVC) measured by a spirometry. Safety was evaluated by the adverse events reported. Efficacy was measured by comparing the initial and final FVC values. Data analysis was performed using the statistical package Excel for Windows 2010.
Results A total of 64 IPF patients were included (55 male, mean age 69 years): 43 in treatment with pirfenidone and 21 with nintedanib. The median duration of treatment (months) was 11 (3–47) with pirfenidone and 19 (3–46) with nintedanib. Patients with pirfenidone: 16 had no previous treatment; the mean FVC initial and final value was 72.7% and 79% respectively; one patient needed a dose reduction to control side effects; three patients suffered an exacerbation since pirfenidone initiation; most frequent adverse events were dyspnea (n=20), dry cough (n=12) and photosensitivity (n=7). Patients with nintedanib: eight had received pirfenidone before nintedanib; the mean FVC initial and final value was 71.6% and 69.4% respectively; one patient discontinued treatment for intolerance; two patients suffered an exacerbation since nintedanib initiation; and most frequent adverse events were diarrhoea (n=11), weight loss (n=7) and increase of the glutamic transaminase (n=6).
Conclusion Patients treated with pirfenidone improved their FVC, but they experienced more adverse events. Nintedanib stabilised the spirometric profile and was tolerated better than pirfenidone. Although they do not result in a significant FVC elevation and they have an important side-effect profile, both antifibrotics provide a treatment alternative for many patients with IPF.
References and/or acknowledgements To my colleagues.
No conflict of interest.