Nintedanib for idiopathic pulmonary fibrosis (IPF): Data from the German compassionate use program (CUP)

Abstract

Background: Nintedanib is approved for the treatment of IPF and has been shown to slow disease progression by reducing annual lung function decline. Aim: To evaluate the efficacy/tolerability of nintedanib in clinical practice using data from a large cohort of patients treated in the German CUP. Methods: Patients (≥40 years) were required to have a confirmed diagnosis of IPF, forced vital capacity (FVC) ≥50% predicted (pred.) and a carbon monoxide diffusing capacity (D LCO ) 30%-79% pred. and not to be eligible for pirfenidone treatment. Clinical data, pulmonary function tests and adverse events were recorded. Stable disease was defined as Results: Sixty-two patients (48 male/14 female) with moderate IPF (FVC 64±17% pred. and D LCO 40±10% pred.) were treated with nintedanib at 9 centres. 77% of patients switched from pirfenidone (mean treatment duration 14±2 months) mostly due to disease progression (mean decline in FVC 7.4±3% pred. in the 6 months prior to nintedanib intake). Initiation of nintedanib treatment occurred 69±29 months after IPF diagnosis and mean treatment duration was 8±4 months. Most patients (63%) stabilized 6 months after treatment start (mean FVC decline 3±1% vs. -17±2% in patients with disease progression; p Conclusion: Nintedanib was generally well-tolerated and was associated with FVC stabilization in the majority of patients in this real-life setting. Our findings are in agreement with the previously published data.