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Publications

Journal Article Systematic Review Meta-Analysis Review Epublish

PubMed: 42930051 DOI: 10.3390/arm94050073

Efficacy and Safety of Inhaled Treprostinil in Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis.

Adv Respir Med 94(5)

Authors: Jyoti Bajpai, Akshyaya Pradhan, Gaurav Manek, Shivani Maurya, Surya Kant, Marco Alfonso Perrone

Affiliations

  • Department of Respiratory Medicine, King George's Medical University, Lucknow 226003, Uttar Pradesh, India.
  • Department of Cardiology, King George's Medical University, Lucknow 226003, Uttar Pradesh, India.
  • Pulmonary and Critical Care Medicine, MedStar Medical Group, Clinton, MD 20735, USA.
  • Department 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.

MeSH

Idiopathic Pulmonary Fibrosis, Mortality, Lung, Hypertension, Pulmonary, Safety, Fibrosis, Pulmonary Fibrosis, Records, Lung Diseases, Vital Capacity