Antifibrotic therapy in IPF patients with severe physiologic impairment

Rational Patients with idiopathic pulmonary fibrosis (IPF) demonstrate a wide range of lung function impairment at presentation. The efficacy of antifibrotics is largely unknown in patients with more advanced disease due to their exclusion from clinical trials. We sought to investigate the outcomes on antifibrotic therapies in a cohort of IPF patients with more advanced lung function impairment, defined as percent predicted forced vital capacity (%FVC) <50% and/or percent predicted carbon monoxide diffusing capacity (%Dlco) < 30% in a tertiary ILD care center. Patients and methods We performed a retrospective chart/database review of patients with IPF evaluated in the Inova Advanced Lung Disease Program who presented for the period from January 2011 to February 2019. All patients with FVC ≤ 50% predicted and/or a DLco ≤ 30% predicted obtained at first consultation or during follow-up were included in this analysis. Demographic, functional and treatment characteristics as well as outcome data were collated. Results On the 554 patients with IPF evaluated in our center during that period, 170 (31%) met the inclusion criteria of physiologic impairment at their first presentation, and a further 72 (13%) during follow-up. Of these 242 patients, 56 patients were managed before October 2014. Of the 186 patients seen after this date, 163 (89%) were placed on antifibrotic therapies including 87 who were treated with pirfenidone (53%), 45 with nintedanib (28%) and 31 with both sequentially (19%). Mean duration of treatment was 18 ± 15 months (range 0-54 months). Figure 1 provides a Kaplan-Meier analysis of transplant-free survival in patients treated with antifibrotics versus treatment-naïve historical controls seen prior to October 2014. Conclusion Patients with severe physiologic impairment treated with antifibrotic therapy have significantly better outcomes. Antifibrotic therapy should be offered to patients who present with or develop more severe disease. These results also support the inclusion of more severe patients in future clinical trials of novel IPF therapies. (Figure Presented) .