Open Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321/ BUILD 3 / NCT00391443

Study Overview
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Brief Summary
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This Open-label extension study in patients with Idiopathic Pulmonary Fibrosis who completed protocol AC-052-321 / BUILD 3 (NCT00391443) will asses the long term safety and tolerability of bosentan in patients with idiopathic pulmonary fibrosis (IPF).

Official Title
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Open-Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321 (NCT00391443)

Conditions
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Idiopathic Pulmonary Fibrosis

Intervention / Treatment
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* Drug: Bosentan


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients should have completed all the assessments from the BUILD 3 (NCT00391443) end of study (EOS) visit. Signed informed consent prior to initiation of any study-related procedures. Women of childbearing potential must have a negative serum pregnancy test and use reliable methods of contraception during study treatment and for 3 months after study treatment termination. Exclusion Criteria: Any major violation of protocol AC-052-321 / BUILD 3 (NCT00391443). Pregnancy or breast-feeding. AST and/or ALT > 3 times the upper limit of the normal range. Any known factor or disease that might interfere with treatment compliance, study conduct or interpretation of the results, such as drug or alcohol dependence or psychiatric disease. Known hypersensitivity to bosentan or any of the excipients.

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Non-Randomized
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: 1<br>For patients who were administered bosentan during BUILD 3 (NCT00391443): Same dose will continue For patients who were administered placebo during BUILD 3 (NCT00391443): Initial dose: 62.5 mg for 4 weeks Maintenance dose: 125 mg | Drug: Bosentan<br>* For patients who were administered Bosentan during BUILD 3 (NCT00391443): continue on same dose For patients who were administered placebo during BUILD 3 (NCT00391443): Oral Bosentan 62.5 mg for 4 weeks; maintenance dose: 125 mg ( 62.5 if patient weighs < 90 lbs.)<br>* Other names: Tracleer;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Extent of Exposure to Bosentan in Patients With Idiopathic Pulmonary Fibrosis (IPF) | Mean extent of exposure to bosentan treatment in months | Start of study to end of study, up to 21 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Number of Patients Exposed to Bosentan Over Time | Numbers of participants exposed to bosentan treatment over time | Start to end of study, up to 21 months | 
| Adverse Events (AE) Leading to Discontinuation of Study Drug. | Number of participants with at least one AE that led to permanent discontinuation of study treatment. | Start to end of study, up to 21 months | 
| Treatment-emergent Serious Adverse Events (SAE) | Number of participants with at least one SAE during the study. | up to 21 months plus 28 days after the end of study drug | 
| Occurrence of Liver Function Test (LFT: Alanine Aminotransferase (ALT) and Aspartate Aminotransferase (AST)) Abnormality. | Number of participants with an increase in ALT and/or AST to > 3 times upper limit of normal during the study. | up to 21 months, plus 24 hours after the end of study treatment | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Actelion, Idiopathic Pulmonary Fibrosis, bosentan, Tracleer, Interstitial Lung Disease, BUILD 3 (NCT00391443)