Related Experiment Video
Updated: Jun 13, 2026

Unilateral Lung Volume Analysis Using Micro-CT for Enhanced Assessment of Pulmonary Fibrosis in Preclinical Models
Published on: June 20, 2025
Evaluation of bosentan for idiopathic pulmonary fibrosis
Jeffrey J Swigris1, Kevin K Brown
1Interstitial Lung Disease Program, National Jewish Medical and Research Center, 1400 Jackson Street, Denver, CO, USA. swigrisj@njc.org
Abstract:
Idiopathic pulmonary fibrosis (IPF) is a quality-of-life-altering and life-shortening lung disease manifested by physiologic restriction, hypoxemia and progressive shortness of breath. Despite nearly 30 years of investigation, the median survival for patients with this disease remains dismal at approximately 3 years from the time of diagnosis. Recent investigations have identified a number of potential molecular therapeutic targets for IPF that include endothelin-1 and other fibrogenic cytokines. Bosentan, a nonselective endothelin receptor antagonist approved in the USA and Europe for the treatment of patients with pulmonary arterial hypertension, is currently undergoing evaluation as a potential therapy for IPF. A recently completed multinational, placebo-controlled trial failed to show a beneficial impact of bosentan on the primary end point, but results from a hypothesis-generating, post hoc analysis of data from this trial have prompted an assessment of the drug for efficacy in a selected subgroup of IPF patients - those with biopsy-proven IPF and little radiographic honeycombing. Results from this trial are anticipated in 2009.
Insights
Idiopathic pulmonary fibrosis (IPF) is a severe lung disease with poor survival. Bosentan, an endothelin receptor antagonist, showed no overall benefit in a recent IPF trial but may benefit a specific patient subgroup.
Area of Science:
- Pulmonology
- Pharmacology
- Fibrotic lung diseases
Background:
- Idiopathic pulmonary fibrosis (IPF) is a progressive, life-limiting lung disease with a median survival of approximately 3 years.
- Current therapeutic options for IPF are limited, highlighting the need for novel treatment strategies.
- Endothelin-1 and fibrogenic cytokines are implicated as potential molecular targets in IPF pathogenesis.
Purpose of the Study:
- To evaluate the efficacy of bosentan, a nonselective endothelin receptor antagonist, as a potential therapy for idiopathic pulmonary fibrosis (IPF).
Main Methods:
- A multinational, placebo-controlled clinical trial was conducted to assess bosentan's impact on IPF.
- The trial's primary endpoint was evaluated, and a post hoc analysis identified a specific subgroup for further assessment.
- The selected subgroup comprised patients with biopsy-proven IPF and minimal radiographic honeycombing.
Main Results:
- The primary endpoint of the multinational trial did not demonstrate a beneficial impact of bosentan in patients with IPF.
- A post hoc analysis suggested potential efficacy in a selected subgroup of IPF patients.
- Results from the ongoing assessment in this subgroup are anticipated.
Conclusions:
- Bosentan did not show overall efficacy in the studied IPF patient population.
- Further investigation is warranted to determine the potential benefit of bosentan in specific IPF subgroups, such as those with biopsy-proven disease and limited honeycombing.
Related Concept Videos
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme (ECE). Of...
Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies
Medical History
Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care
Pulmonary Embolism I: Introduction
Pulmonary Embolism I: Introduction
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...