A large proportion of patients with idiopathic pulmonary fibrosis (IPF) who receive reduced-dose antifibrotic agents experience improved survival compared with cessation of treatment, according to the results of a retrospective analysis published in the journal Respiratory Medicine.

It is well known that IPF is a severe interstitial lung disease for which 2 effective antifibrotic agents—nintedanib and pirfenidone—are available. Although many patients need to receive a decreased dosage or pause treatment with antifibrotic agents because of adverse events, the impact of treatment reduction remains uncertain.

The investigators sought to explore the impact of antifibrotic treatment reduction on death in a large, real-life IPF cohort. They conducted a review of medical data derived from patients included in the Danish Pulmonary Fibrosis Biomarker (PFBIO) cohort study.

Continue Reading

The primary study endpoint was time until death from any cause. The observation period began at the time of IPF diagnosis and ended with death from any cause or censoring.

Learn more about idiopathic pulmonary fibrosis

In the current analysis, 5 patient groups were defined according to type of antifibrotic agent (nintedanib or pirfenidone) and intensity of treatment (full, reduced, or no treatment): (1) no treatment group; (2) nintedanib full treatment group; (3) pirfenidone full treatment group; (4) nintedanib reduced treatment group; and (5) pirfenidone reduced treatment group.

Three hundred seventy-five patients from the Danish PFBIO study were followed between April 2016 and November 2021, with a median follow-up time of 1.84 years. None of the patients in the cohort had undergone lung transplantation. Overall, 106 participants were treated with nintedanib, 221 were treated with pirfenidone, and 48 received no treatment.

Among individuals treated with nintedanib and pirfenidone, 80.19% and 67.42%, respectively, received reduced treatment when the entire follow-up period was taken into account.

Results of the study showed that treatment with nintedanib and pirfenidone was associated with significantly improved survival, compared with no antifibrotic therapy, independent of treatment intensity (hazard ratio [HR], 0.31; 95% CI, 0.19-0.53; P <.001 with nintedanib vs HR, 0.26; 95% CI, 0.16-0.42; P <.001 with pirfenidone). In lower intensities, neither nintedanib nor pirfenidone was associated with worse survival outcomes.

The authors concluded that, “…both nintedanib and pirfenidone independent of treatment intensity were associated with improved survival, indicating that dose reduction appears to be a good alternative if full treatment is not tolerated.”


Porse S, Hoyer N, Shaker SB. Impact of reduction in antifibrotic treatment on mortality in idiopathic pulmonary fibrosis. Respir Med. 2022;204:107015. doi:10.1016/j.rmed.2022.107015