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

Actelion132 enrolled

Overview

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.

Study Type

INTERVENTIONAL

Allocation

RANDOMIZED

Purpose

TREATMENT

Masking

DOUBLE

Enrollment

132

Conditions

Pulmonary Fibrosis Scleroderma, Systemic

Interventions

BosentanDRUG

Eligibility

Sex: ALLMin age: 18 Years

Medical Language ↔ Plain English

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)

Outcomes

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

Data from ClinicalTrials.gov

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