Real life experience with nintedanib in patients with idiopathic pulmonary fibrosis

Background: Idiopathic pulmonary fibrosis (IFP) is a chronic progressive fibrosing interstitial lung disease associated with high mortality and a high burden of disease. Nintedanib, an oral tyrosine kinase inhibitor has been shown to slow down disease progression in 2 randomised placebo-controlled trials by reducing annual decline in forced vital capacity (FVC). However, real-life experience is limited. Methods: Nintedanib was initiated in IPF patients until 02/2015. Lung function parameters, adverse events, and treatment compliance were assed longitudinally. Disease progression was defined as decrease of FVC ≥10% and/or diffusion capacity of the lung (DLCO) ≥15%. Results: Nintedanib was initiated in 60 patients with a mean age of 71 years, 90% male, 69% ex-smoker, baseline FVC of 70% and DLCO of 41% predicted. Cardiovascular comorbidities were present in 41%. Mean time on drug was 11 months (0-40). Nintedanib therapy was continued in 97% and terminated in 2 patients due to progression or side effects. In 4 cases a temporary dose reduction or interruption due to side effects (diarrhoea) was required, in 2 cases nintedanib had to be continued in a reduced dosage. 6 Patients had an acute exacerbation while on treatment, 4 patients died during the observation period due to IPF. Main side effects were diarrhoea (19%); one patient had an acute pulmonary embolism after 5-month on treatment. In 46% a concomitant treatment with anticoagulants was required; no bleeding or cardiac events were observed. Mean decline in FVC was 4.4% and 1.2% DLCO. Conclusion: Nintedanib seems to be an effective treatment option with tolerable side effects in the real life setting, however longer observation is required.