Efficacy and Safety of Oral Bosentan in Pulmonary Fibrosis Associated With Scleroderma

Clinical and experimental studies suggest that bosentan could delay the progression of interstitial lung disease (ILD) associated with systemic sclerosis (SSc), a condition for which no established efficacious treatment is available. The present trial investigates a possible use of oral bosentan, which is currently approved for the treatment of symptoms of pulmonary arterial hypertension (PAH) WHO Class III and IV, to a new category of patients suffering from ILD associated with SSc.

Completed

Looking for future studies?

Notify Me

Key information

Conditions

Pulmonary Fibrosis Connective Tissue Diseases Fibrosis Lung Diseases Lung Diseases, Interstitial Pathologic Processes Pathological Conditions, Signs and Symptoms Respiratory Tract Diseases Scleroderma, Diffuse Scleroderma, Systemic Skin Diseases Skin and Connective Tissue Diseases

Age range

18 year and older

Sex eligibility

All sexes

Study type

Interventional

Phase

Phase 2 / Phase 3

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Main inclusion criteria:

Systemic Sclerosis diffuse or limited
Significant Interstitial Lung Disease on HRCTscan
DLco < 80% predicted
Dyspnea on exertion
Walk not limited by musculoskeletal reasons

Main exclusion criteria:

Interstitial Lung Disease due to other conditions than SSc
End stage restrictive or obstructive lung disease
Severe cardiac or renal diseases
Significant pulmonary arterial hypertension
Smoker (> 5cig./day)
Treatment with immunosuppressive, antifibrotic drugs, high dose corticosteroids (within 4 weeks of randomization)

Treatment and study plan

Bosentan

Drug

Primary outcomes

Change from baseline to End-of-Study in 6-minute walk distance.

Secondary outcomes

Time to death (all causes) or to worsening of PFTs up to End-of-Study.
Worsening of PFTs (on 2 consecutive tests at least 4 weeks apart) is defined as: decrease from baseline ≥ 10% in FVC OR decrease from baseline ≥ 15% in DLco AND ≥ 6% in FVC

Sponsors and collaborators

Lead sponsor

Actelion

Industry

Registry information

Official study title

A Double-blind, Randomized, Placebo-controlled, Multicenter Study to Assess the Efficacy, Safety and Tolerability of Bosentan in Patients With Interstitial Lung Disease Associated With Systemic Sclerosis

Important dates

Study start: 2003
Primary completion: 2005
Study completion: 2005
First posted: Oct 8, 2003
Registry last updated: Feb 3, 2025

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

Published trials that share one or more normalized conditions with this study.

Completed