Survival and Course of Lung Function in the Presence or Absence of Antifibrotic Treatment in Patients with Idiopathic Pulmonary Fibrosis: Long-Term Results of the INSIGHTS-IPF Registry

While antifibrotic therapy is approved for idiopathic pulmonary fibrosis (IPF) based on clinical trials, we aimed to assess the effects of such treatment under real life conditions. We analysed data from a non-interventional, prospective cohort study of consecutively enrolled IPF patients in 20 ILD expert centers in Germany. Propensity scores were applied to account for known differences in baseline characteristics of patients with and without antifibrotic therapy. In 588 patients suitable for analysis, mean age was 69.8 ± 9.1 years, 81.0% were males. Mean duration of IPF since diagnosis was 1.8 ± 3.4 years. The mean % of predicted at baseline was 68.6 ± 18.8 for forced vital capacity (FVC) and 37.8 ± 35.4 for diffusion capacity (DLCO). During a mean follow-up of 1.2 ± 0.7 years, 194 (33.0%) patients died. The 1-year and 2-year survival was 92% vs. 78% and 76% vs. 63% for patients with vs. without antifibrotic therapy (Figure). The risk of death was 37% lower in patients with antifibrotic therapy (Hazard Ratio 0.63, 95%CI: 0.45-0.87; p=0.005). The result was robust (and remained statistically significant) on multivariable analysis. Patients on antifibrotic therapy did not statistically significantly differ in the decline of FVC and DLCO compared to patients without such treatment.