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Multicenter Study
. 2023 Apr;45(4):306-315.
doi: 10.1016/j.clinthera.2023.03.003. Epub 2023 Mar 28.

Effect of Antifibrotic Therapy on Survival in Patients With Idiopathic Pulmonary Fibrosis

Joao A de Andrade  1 , Megan L Neely  2 , Anne S Hellkamp  2 , Daniel A Culver  3 , Hyun J Kim  4 , Timothy Liesching  5 , Leonard J Lobo  6 , Murali Ramaswamy  7 , Zeenat Safdar  8 , Shaun Bender  9 , Craig S Conoscenti  9 , Thomas B Leonard  9 , Scott M Palmer  2 , Laurie D Snyder  2 ; IPF-PRO Registry investigators
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Free article
Multicenter Study

Effect of Antifibrotic Therapy on Survival in Patients With Idiopathic Pulmonary Fibrosis

Joao A de Andrade et al. Clin Ther. 2023 Apr.
Free article

Abstract

Purpose: Real-world studies have reported reduced mortality in patients with idiopathic pulmonary fibrosis (IPF) treated with antifibrotic therapy; however, the initiation or discontinuation of therapy during these studies may have introduced bias. This study investigated the effect of antifibrotic therapy on mortality and other outcomes in patients with IPF using causal inference methodology.

Methods: Data from a multicenter US registry of patients with IPF were used to assess the effect of antifibrotic therapy (nintedanib or pirfenidone) on death, death or lung transplant, respiratory-related hospitalization, and acute worsening of IPF (defined as any health care encounter deemed due to acute worsening of IPF). This study used the Gran method, which accounts for differences in patient characteristics and for treatment initiations and discontinuations during follow-up. The analysis cohort was limited to patients who started antifibrotic therapy on or after the day of enrollment or had never taken it.

Findings: Among the 499 patients analyzed, 352 (70.5%) received antifibrotic therapy. Estimated event rates of death at 1 year were 6.6% (95% CI, 6.1-7.1) for treated patients and 10.2% (95% CI, 9.5-10.9) for control patients. There was a numerical reduction in the risk of death (hazard ratio [HR], 0.53; 95% CI, 0.28-1.03; P = 0.060) but numerical increases in risks of respiratory-related hospitalization (HR, 1.88; 95% CI, 0.90-3.92; P = 0.091) and acute worsening of IPF (HR, 1.71; 95% CI, 0.36-8.09; P = 0.496) in treated versus control patients.

Implications: Analyses based on causal inference methodology suggest that patients with IPF who receive antifibrotic therapy have improved survival.

Keywords: Disease progression; Idiopathic pulmonary fibrosis; Interstitial fibrosis; Mortality.

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Conflict of interest statement

Declaration of Interest JAdeA is a member of the Steering Committee for the IPF-PRO/ILD-PRO Registry; has received speaker fees from Boehringer Ingelheim (BI); has served on a Data Safety Monitoring Board for Respivant Sciences, Roche/Genentech, the National Institutes of Health; and is a Scientific Advisory Committee member for the Pulmonary Fibrosis Foundation. MLN, ASH, LS and SMP are employees of DCRI, which receives funding support from BIPI to coordinate the IPF-PRO/ILD-PRO Registry. In addition, SMP has received research funding paid to Duke/DCRI from AstraZeneca, Bristol Myers Squibb, CareDx; royalties from UpToDate; and speaker fees from Altavant Sciences and Bristol Myers Squibb. DAC is a member of the Steering Committee and Publication Committee for the IPF-PRO/ILD-PRO Registry and reports consulting and speaker fees from BI and Genentech. HJK is a member of the Publication Committee for the IPF-PRO/ILD-PRO Registry and has received a grant for a Patient Education Day from Roche/Genentech. TL, LJL, MR have no disclosures. ZS reports consulting fees and honoraria for lectures from BI and Genentech. SB was an employee of BIPI at the time that this study was conducted. TBL and CSC are employees of BIPI.

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