Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Observational Study
. 2023 Aug;201(4):335-343.
doi: 10.1007/s00408-023-00628-4. Epub 2023 Jun 21.

Improved Survival of IPF patients Treated With Antifibrotic Drugs Compared With Untreated Patients

Mark G J P Platenburg  1 , Coline H M van Moorsel  2   3 , Ivo A Wiertz  2 , Joanne J van der Vis  2   4 , Adriane D M Vorselaars  2   3 , Marcel Veltkamp  2   3 , Jan C Grutters  2   3
Affiliations
Observational Study

Improved Survival of IPF patients Treated With Antifibrotic Drugs Compared With Untreated Patients

Mark G J P Platenburg et al. Lung. 2023 Aug.

Abstract

Purpose: Pirfenidone and nintedanib unequivocally inhibit FVC decline, but have been inconsistently linked to reduced mortality in phase III studies. On the contrary, real-world data show a survival benefit of antifibrotic drugs. However, it is unknown what this benefit is across different Gender, Age, and Physiology (GAP) stages.

Research questions: Is there a difference in transplant-free (TPF) survival of IPF patients receiving antifibrotic drugs (IPFAF) compared with an untreated cohort (IPFnon-AF)? Is this different for patients with GAP stage I, II, or III.

Methods: This is a single-center observational cohort study using prospectively included patients diagnosed with IPF between 2008-2018. Primary outcomes were TPF survival difference and 1-, 2-, and 3-year cumulative mortality for IPFAF and IPFnon-AF. This was repeated after stratification for GAP stage.

Results: In total, 457 patients were included. The median transplant-free survival was 3.4 years in IPFAF (n = 313) and 2.2 years in IPFnon-AF (n = 144, p = 0.005). For GAP stage II, a median survival of 3.1 and 1.7 years was noted for IPFAF (n = 143) and IPFnon-AF (n = 59, p < 0.001), respectively. A significantly lower 1-, 2-, and 3- year cumulative mortality was found for IPFAF with GAP stage II (1 yr: 7.0% vs 35.6%, 2 yr: 26.6% vs 55.9%, and 3 yr: 46.9% vs 69.5%). The 1-year cumulative mortality of IPFAF with GAP III was also significantly lower (19.0% vs 65.0%).

Conclusion: This large real-world study showed a survival benefit in IPFAF compared with IPFnon-AF. This especially holds true for patients with GAP stage II and III.

Keywords: Antifibrotic drugs; GAP; IPF; Nintedanib; Pirfenidone; Survival.

PubMed Disclaimer

References

    1. Raghu G, Collard HR, Egan JJ et al (2011) An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 183(6):788–824 - DOI - PubMed - PMC
    1. Ley B, Collard HR, King TE (2011) Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 183(4):431–440 - DOI - PubMed
    1. King TE, Bradford WZ, Castro-Bernardini S et al (2014) A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 370(22):2083–2092 - DOI - PubMed
    1. Noble PW, Albera C, Bradford WZ et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): Two randomised trials. Lancet. 2011;377(9779):1760–9. Available from: http://dx.doi.org/ https://doi.org/10.1016/S0140-6736(11)60405-4
    1. Richeldi L, Costabel U, Selman M et al (2011) Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med. 365(12):1079–1087. https://doi.org/10.1056/NEJMoa1103690 - DOI - PubMed

Publication types

MeSH terms

Substances

LinkOut - more resources