Efficacy and Safety of Inhaled Treprostinil in Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis.
4 affiliations
- 1Department of Respiratory Medicine, King George's Medical University, Lucknow 226003, Uttar Pradesh, India.
- 2Department of Cardiology, King George's Medical University, Lucknow 226003, Uttar Pradesh, India.
- 3Pulmonary and Critical Care Medicine, MedStar Medical Group, Clinton, MD 20735, USA.
- 4Department of Cardiology, University of Rome Tor Vergata, 00133 Rome, Italy.
Abstract
Background
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic interstitial lung disease with high morbidity and mortality. Inhaled treprostinil showed pulmonary vascular and lung-function signals in pulmonary hypertension associated with Interstitial lung disease (PH-ILD) and was subsequently tested in IPF trials.Objectives
This systematic review and meta-analysis assessed the efficacy and safety of inhaled treprostinil in adults with IPF.Methods
PubMed, Google Scholar and the Cochrane Library/CENTRAL were searched for records published from January 2000 to 2026. A random-effects meta-analysis used the DerSimonian-Laird tau squared and inverse-variance weighting methods. Continuous outcomes were pooled as generic inverse-variance effects from reported estimates and 95% confidence intervals. Hazard ratios were pooled on the log scale. Safety outcomes were pooled as risk ratios from event counts.Results
The searches identified 1552 records. After deduplication, 379 records were screened and 85 full-text reports were assessed. Eleven reports were included in the systematic review. TETON-1 and TETON-2 were the two direct-IPF randomized trials within the coordinated TETON program eligible for primary meta-analysis. Inhaled treprostinil improved absolute forced vital capacity (FVC) change at 52 weeks compared with placebo (MD 111.28 mL, 95% CI 77.61 to 144.95, p < 0.001; I squared 8.5%). Clinical worsening was reduced (HR 0.69, 95% CI 0.57 to 0.84, p < 0.001; I squared 0.0%). Acute exacerbation was lower in pooled analysis (HR 0.52, 95% CI 0.30 to 0.91, p = 0.022), while all-cause mortality did not differ significantly (HR 0.70, 95% CI 0.47 to 1.05, p = 0.086). Adverse-event discontinuation, cough and headache were more frequent with treprostinil.Conclusions
In direct IPF randomized evidence, inhaled treprostinil preserved lung function and reduced clinical worsening at 52 weeks.
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BibTeX
@article{bajpai2026efficacy,
title = {{Efficacy and Safety of Inhaled Treprostinil in Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis.}},
author = {Jyoti Bajpai and Akshyaya Pradhan and Gaurav Manek and Shivani Maurya and Surya Kant and Marco Alfonso Perrone},
journal = {Advances in respiratory medicine},
year = {2026},
month = oct,
doi = {10.3390/arm94050073},
url = {https://europepmc.org/article/MED/42930051},
note = {CC BY},
}
RIS
TY - JOUR
TI - Efficacy and Safety of Inhaled Treprostinil in Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis.
AU - Jyoti Bajpai
AU - Akshyaya Pradhan
AU - Gaurav Manek
AU - Shivani Maurya
AU - Surya Kant
AU - Marco Alfonso Perrone
PY - 2026
DA - 2026/10/08
JO - Advances in respiratory medicine
DO - 10.3390/arm94050073
UR - https://europepmc.org/article/MED/42930051
AB - Background Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic interstitial lung disease with high morbidity and mortality. Inhaled treprostinil showed pulmonary vascular and lung-function signals in pulmonary hypertension associated with Interstitial lung disease (PH-ILD) and was subsequently tested in IPF trials.Objectives This systematic review and meta-analysis assessed the efficacy and safety of inhaled treprostinil in adults with IPF.Methods PubMed, Google Scholar and the Cochrane Library/CENTRAL were searched for records published from January 2000 to 2026. A random-effects meta-analysis used the DerSimonian-Laird tau squared and inverse-variance weighting methods. Continuous outcomes were pooled as generic inverse-variance effects from reported estimates and 95% confidence intervals. Hazard ratios were pooled on the log scale. Safety outcomes were pooled as risk ratios from event counts.Results The searches identified 1552 records. After deduplication, 379 records were screened and 85 full-text reports were assessed. Eleven reports were included in the systematic review. TETON-1 and TETON-2 were the two direct-IPF randomized trials within the coordinated TETON program eligible for primary meta-analysis. Inhaled treprostinil improved absolute forced vital capacity (FVC) change at 52 weeks compared with placebo (MD 111.28 mL, 95% CI 77.61 to 144.95, p < 0.001; I squared 8.5%). Clinical worsening was reduced (HR 0.69, 95% CI 0.57 to 0.84, p < 0.001; I squared 0.0%). Acute exacerbation was lower in pooled analysis (HR 0.52, 95% CI 0.30 to 0.91, p = 0.022), while all-cause mortality did not differ significantly (HR 0.70, 95% CI 0.47 to 1.05, p = 0.086). Adverse-event discontinuation, cough and headache were more frequent with treprostinil.Conclusions In direct IPF randomized evidence, inhaled treprostinil preserved lung function and reduced clinical worsening at 52 weeks.
SN - 2451-4934
SN - 2543-6031
ER -