Use of nintedanib for the treatment of idiopathic pulmonary fibrosis: multi-centre real life experience in Germany

Background: Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease with a poor life expectancy. Nintedanib is a multiple tyrosine kinases inhibitor recently approved for the treatment of IPF, slowing disease progression by reducing the annual decline in lung function. Here we report the results of a large cohort of IPF patients who received nintedanib within a compassionate-use program (CUP) in Germany. Methods: Since May 2014 62 IPF patients were treated with nintedanib inside the CUP in 9 centers across Germany. Patients should have a diagnosis of IPF based upon the ATS/ERS/JRS/ALAT IPF 2011 guideline, age ≥40 years, forced vital capacity (FVC) ≥50% pred. and a carbon monoxide diffusing capacity (DLCO) 30%-79% pred. and should not be eligible for treatment with pirfenidone as judged by the treating physician. Clinical data, pulmonary function tests and adverse events were recorded until July 2015. Results: The patients (48 Male and 14 Female) were 71 ± 8 years old and had a moderate IPF (FVC 62 ± 16% pred. and DLCO 40 ± 10% pred.). 70% of patients had received pirfenidone alone or in combination before treatment initiation. The mean duration of nintedanib intake was 31 ± 8 weeks. At the end of follow-up 65% patients were stable, as defined by a decline in FVC < 5% from baseline and no worsening of symptoms or radiologic findings. 66% of the patients had side effects, mostly diarrhea (61%) and weight loss (57%). Dose reduction occurred in 29% of cases. Nintedanib was definitively discontinued in 4 patients (3 due to side effects, one due to lack of benefit) and temporary in 17 patients. The median duration of discontinuation was 14.5 (range 3 – 22) days. Conclusion Nintedanib intake was generally well tolerated and was associated with stabilization of the majority of patients. Our real life data are in line with the previous published safety and efficacy data on nintedanib as treatment for IPF.