Background: Idiopathic pulmonary fibrosis (IPF) is a progressive disease leading to respiratory failure and death. Antifibrotic therapies (AFT) slow disease progression, but their impact on survival is unknown.  

Aim: to assess survival benefit of AFT and identify predictors of outcome in a real-world IPF cohort.

Methods: A retrospective single-centre study evaluated patient demographics, AFT and lung function data between 2007 to 2020. Patients were stratified according to GAP index. Kaplan-Meier analysis was used to compare survival between treated and untreated IPF cohorts. Predictors of survival were determined by Cox proportional hazard regression analysis.

Results: In total 410 IPF patients (155 untreated and 255 on antifibrotic treated) with a median(IQR) follow up 1053(655-1581) days were included. Use of AFT improved survival (median(IQR)) 1148(1052-1309) vs 1003(797-1188) days for untreated IPF, p=0.04 (Figure 1). In both cohorts, median survival was worse for GAP stage 3. AFT improved survival for GAP stage 2 (untreated 724(654-1119) vs treated 1256(1109-1564) days) and stage 3 (untreated 371(272-649) vs treated 835(675-1109) days). Multivariate Cox regression analysis identified duration of AFT and baseline DLCO as predictors of survival in the treated cohort.

Conclusion: Antifibrotic therapy improves survival in IPF. Baseline DLCO and duration of antifibrotic therapy are the best predictors of outcome.