In the Phase III INPULSIS^® trials, 52 weeks’ treatment with nintedanib reduced decline in forced vital capacity (FVC) versus placebo in patients with idiopathic pulmonary fibrosis (IPF). Patients who completed the INPULSIS^® trials could receive nintedanib in an open-label extension trial (INPULSIS^®-ON). Patients with FVC <50 % predicted were excluded from INPULSIS^®, but could participate in INPULSIS^®-ON. In patients with baseline FVC ≤50 % and >50 % predicted at the start of INPULSIS^®-ON, the absolute mean change in FVC from baseline to week 48 of INPULSIS^®-ON was −62.3 and −87.9 mL, respectively (n = 24 and n = 558, respectively). No new safety signals were identified in INPULSIS^®-ON compared with INPULSIS^®. The decline in FVC in INPULSIS^®-ON in both subgroups by baseline FVC % predicted was similar to that in INPULSIS^®, suggesting that nintedanib may have a similar effect on disease progression in patients with advanced disease as in less advanced disease.