TY - JOUR
T1 - Effect of Antifibrotic Therapy on Survival in Patients With Idiopathic Pulmonary Fibrosis
AU - IPF-PRO Registry investigators
AU - de Andrade, Joao A.
AU - Neely, Megan L.
AU - Hellkamp, Anne S.
AU - Culver, Daniel A.
AU - Kim, Hyun J.
AU - Liesching, Timothy
AU - Lobo, Leonard J.
AU - Ramaswamy, Murali
AU - Safdar, Zeenat
AU - Bender, Shaun
AU - Conoscenti, Craig S.
AU - Leonard, Thomas B.
AU - Palmer, Scott M.
AU - Snyder, Laurie D.
N1 - Publisher Copyright:
© 2023 The Author(s)
PY - 2023/4
Y1 - 2023/4
N2 - 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.
AB - 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.
KW - Disease progression
KW - Idiopathic pulmonary fibrosis
KW - Interstitial fibrosis
KW - Mortality
KW - Humans
KW - Pyridones
KW - Idiopathic Pulmonary Fibrosis/drug therapy
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U2 - 10.1016/j.clinthera.2023.03.003
DO - 10.1016/j.clinthera.2023.03.003
M3 - Article
C2 - 36997445
AN - SCOPUS:85151341315
SN - 0149-2918
VL - 45
SP - 306
EP - 315
JO - Clinical Therapeutics
JF - Clinical Therapeutics
IS - 4
ER -