Efficacy and safety of combined use of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis

Recent studies suggested that combination therapy with pirfenidone and nintedanib has manageable safety and tolerability profile in patients with idiopathic pulmonary fibrosis (IPF). However, data from real-world practice is lacking. We aimed to investigate safety and efficacy of combination therapy in patients with IPF. Clinical data of IPF patients treated with pirfenidone and nintedanib were retrospectively reviewed. Adverse events and lung function declines were compared before and after combination treatment At the beginning of combination therapy, of total 22 patients the mean age was 68.7 years and 86.4% were male. The median follow-up duration was 10.7 months. Majority of patients (95.5%) received add-on nintedanib during treatment with pirfenidone at a stable dose (mean dose: 1773.9 mg/day, median duration: 31 months). Most common adverse events after the combination were diarrhea (63.6%), dyspepsia/abdominal pain (13.6%) and anorexia (9.1%). In 3 patients (15.6%), one of the antifibrotic agents was stopped due to anorexia or dyspepsia. In 16 patients with serial lung function data before and after treatment, decline rate in forced vital capacity (FVC) was significantly reduced (-0.41% [before] vs -0.12% [after] predicted/month, p = 0.045) (Figure 1). Our data suggests that the combination therapy had a manageable safety profile and might be more effective than monotherapy.