Work

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

Year: 2026

Type: review

Study designs: Systematic Review, Meta-Analysis

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.

Source: Advances in respiratory medicine

DOI: 10.3390/arm94050073

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

Institutions: King George's Medical University, MedStar Health, University of Rome Tor Vergata

Language: English

Topic: Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis

Subfield: Pulmonary and Respiratory Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $1,568