Completed

BUILD 1A Double-blind, Randomized, Placebo-controlled, Multicenter Study to Assess the Efficacy, Safety, and Tolerability of Bosentan in Patients With Idiopathic Pulmonary Fibrosis, Open Label Extension

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What is being tested

bosentan

+ Placebo

Drug
Who is being recruted

Fibrosis+4

+ Lung Diseases

+ Pathologic Processes

Over 18 Years
See all eligibility criteria
How is the trial designed

Treatment Study

Placebo-ControlledPhase 2 & 3
Interventional
Study Start: August 2003
See protocol details

Summary

Principal SponsorActelion
Last updated: January 13, 2026
Sourced from a government-validated database.Claim as a partner

Study start date: August 1, 2003

Actual date on which the first participant was enrolled.

Endothelin-1 (ET-1) is expressed in a variety of pulmonary pathological conditions including pulmonary vascular disease and pulmonary fibrosis. Bosentan (an oral dual ET-1 receptor antagonist) could delay the progression of idiopathic pulmonary fibrosis (IPF), a condition for which no established treatment is available. The present trial investigates a possible use of 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 IPF. It was decided to offer Open Label treatment (bosentan) for patients willing to continue in the BUILD 1 study.

Official TitleA Double-blind, Randomized, Placebo-controlled, Multicenter Study to Assess the Efficacy, Safety, and Tolerability of Bosentan in Patients With Idiopathic Pulmonary Fibrosis, Open Label Extension 
NCT00071461
Principal SponsorActelion
Last updated: January 13, 2026
Sourced from a government-validated database.Claim as a partner

Protocol

This section provides details of the study plan, including how the study is designed and what the study is measuring.
Design Details

158 patients to be enrolled

Total number of participants that the clinical trial aims to recruit.

Treatment Study

These studies test new ways to treat a disease, condition, or health issue. The goal is to see if a new drug, therapy, or approach works better or has fewer side effects than existing options.



Eligibility

Researchers look for people who fit a certain description, called eligibility criteria: person's general health condition or prior treatments.
Conditions
Criteria

Any sex

Biological sex of participants that are eligible to enroll.

Over 18 Years

Range of ages for which participants are eligible to join.

Healthy volunteers not allowed

If individuals who are healthy and do not have the condition being studied can participate.

Conditions

Pathology

FibrosisLung DiseasesPathologic ProcessesPulmonary FibrosisRespiratory Tract DiseasesLung Diseases, InterstitialIdiopathic Pulmonary Fibrosis

Criteria

Inclusion Criteria: 1. Male or female patients over 18 years of age. * Women must be either postmenopausal (i.e., amenorrhea for at least 1 year), or surgically or naturally sterile. * Women of childbearing potential must have a negative pre-treatment pregnancy test and use a reliable method of contraception during study treatment and for at least 3 months after study treatment termination. 2. IPF proven diagnosis \< 3 years documented according to ATS/ERS international multidisciplinary consensus, with or without surgical (thoracoscopic or open) chest lung biopsy 3. Duration of illness ≥ 3 months. 4. Six-minute walk test distance (limited by dyspnea) ≥ 150 meters and \< 500 meters 5. Patients who have signed the informed consent form prior to initiation of any study procedure. Exclusion Criteria: 1. Interstitial lung disease due to conditions other than IPF, including but not limited to radiation, sarcoidosis, hypersensitivity pneumonitis, bronchiolitis obliterans with organizing pneumonia, and cancer. 2. History of clinically significant environmental exposure known to cause pulmonary fibrosis (drugs, asbestos, beryllium, radiation, domestic birds, etc.). 3. Severe concomitant illness limiting life expectancy (\< 1 year). 4. FVC ≥ 90% predicted. 5. Severe restrictive lung disease: FVC \< 50% predicted or FVC \< 1.2 l, or DLco \< 30% predicted or residual volume ≥ 120% predicted. 6. Severe obstructive lung disease: FEV1/FVC\< 0.65. 7. Documented improvement of patient's condition within 12 months prior to randomization with or without IPF-specific therapy (e.g., corticosteroids, immunosuppressive, cytotoxic or antifibrotic drugs, TNFa blocker, interferon g). 8. Recent pulmonary or upper respiratory track infection (within 4 weeks of randomization). 9. PaO2 \< 55 mm Hg (sea level) or 50 mm Hg (altitude) at rest on room air. 10. Echocardiographic evidence of severe pulmonary hypertension (PH): systolic pulmonary pressure ≥ 50 mm Hg or tricuspid regurgitation velocity ≥ 3.2 m/sec (unless severe PH is invalidated by a right heart catheterization). If the pulmonary pressure is not quantifiable, presence of significant right ventricular enlargement or hypertrophy or right ventricular dysfunction. 11. Severe chronic heart failure, e.g., NYHA class III or IV and/or left ventricular ejection fraction \< 25%. 12. Acute or chronic impairment (other than dyspnea) limiting the ability to comply with study requirements, e.g., the 6MWT or the PFTs. (e.g., angina pectoris, intermittent claudicating, chronic arthritis). 13. Baseline values of liver transaminases, i.e., aspartate aminotransferases (AST) and/or alanine aminotransferases (ALT) \> 3 times the upper limit of normal ranges. 14. Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C. 15. Serum creatinine ≥ 2.5 mg/dl (221 mmol/l) or dialysis. 16. Hemoglobin concentration \< 75% the lower limit of normal ranges. 17. Systolic blood pressure \< 85 mm Hg. 18. Pregnancy or breast-feeding. 19. Current drug or alcohol dependence. 20. Smoker (≥ 5 cigarettes per day) or former smoker (≥ 5 cigarettes per day) having stopped less than 6 months prior to randomization. 21. Recently started (\< 8 weeks from Screening visit) or planned cardio-pulmonary rehabilitation program based on exercise. 22. Treatment with oral corticosteroids (\> 15 mg/day prednisone or equivalent), immunosuppressive, cytotoxic or antifibrotic drugs such as TNF alpha blocker, or interferon gamma within 4 weeks of randomization.within 4 weeks of randomization. 23. Treatment with glibenclamide (glyburide), cyclosporine A or tacrolimus within 1 weeks of randomization. 24. Treatment with an endothelin receptor antagonist within 3 months of randomization. 25. Treatment within 3 months of randomization or planned treatment with another investigational drug. 26. Known hypersensitivity to bosentan or any of the excipients.

Study Plan

Find out more about all the medication administered in this study, their detailed description and what they involve.
Treatment Groups
Study Objectives

2 intervention groups are designated in this study

50% chance of being blinded to the placebo group

Treatment Groups

Group I

Experimental
Initial dose: 62.5 mg b.i.d. for 4 weeks. * Target dose: - body weight \> 40 kg (90 lb): 125 mg b.i.d., (if the initial dose is well tolerated). * body weight \< 40 kg (90 lb): 62.5 mg b.i.d.

Group II

Placebo
Initial dose: 62.5 mg b.i.d. for 4 weeks. * Target dose: - body weight \> 40 kg (90 lb): 125 mg b.i.d., (if the initial dose is well tolerated). * body weight \< 40 kg (90 lb): 62.5 mg b.i.d.

Study Objectives

Primary Objectives

Secondary Objectives

Study Centers

These are the hospitals, clinics, or research facilities where the trial is being conducted. You can find the location closest to you and its status.

This study has 29 locations

Suspended

University of Alabama at Birmingham - Pulmonary Division

Birmingham, United StatesSee the location
Suspended

David Geffen School of Medicine at UCLA - Division of Pulmonary and Critical Care Medicine

Los Angeles, United States
Suspended

UCSD Medical Center

San Diego, United States
Suspended

University of California - Ambulatory Care Center

San Francisco, United States
Completed29 Study Centers