Idiopathic Pulmonary Fibrosis (IPF) MedDRA version: 8.1 Level: LLT Classification code 10021240 Term: Idiopathic pulmonary fibrosis
Conditions
Sponsors
ACTELION PHARMACEUTICALS LTD
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: *Signed informed consent. *Male or female patients aged 18 years or older (females of child-bearing potential must have been surgically sterilized or use a reliable method of contraception). *Proven diagnosis of IPF according to ATS/ERS statement, of =65 years) yes F.1.3.1 Number of subjects for this age range
Exclusion criteria
Exclusion criteria: *Interstitial lung disease due to conditions other than IPF. *Presence of extensive honeycomb (HC) on Baseline high-resolution computed tomography (HRCT) scan. The patient is not allowed in BUILD 3 if HC involves more than 5 % of the parenchyma in 3 or more of the 6 zones (i.e., right and left lung, viewed at the levels of tracheal carina, inferior pulmonary veins, and 1 cm above the dome of the diaphragm), whether the involvement is unilateral or bilateral. *Severe concomitant illness limiting life expectancy ( 1.5 times the upper limit of the normal ranges (ULN). *Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C. *Serum creatinine = 2.5 mg/dl (221 mmol/l) or chronic dialysis. *Hemoglobin concentration 20 mg/day of prednisone or equivalent), -Immunosuppressive or cytotoxic drugs, -Antifibrotic drugs including pirfenidone, D-penicillamine, colchicine, TNFa blocker, imatinib, interferon g, cyclophosphamide, azathioprine, -Chronic use of N-acetylcysteine (prescribed for IPF). *Oral anticoagulants other than those indicated for a venous or arterial thrombotic disease. *Treatment with glibenclamide (glyburide) and calcineurin inhibitors (cyclosporine A, tacrolimus) up to 1 week prior to randomization. *Treatment with an endothelin receptor antagonist up to 3 months prior to randomization. *Participation in the BUILD 1 trial. *Treatment with another investigational drug up to 3 months prior to randomization or planned treatment. *Known hypersensitivity to bosentan or any of the excipients.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main Objective: - To demonstrate that bosentan delays disease worsening or death in patients with idiopathic pulmonary fibrosis (IPF).;Secondary Objective: - To assess the effects of bosentan on quality of life, dyspnea, and pulmonary function tests (PFTs) in this patient population. -To assess the safety and tolerability of bosentan in this patient population.;Primary end point(s): *Time to occurrence of disease worsening or death up to EOS. -Disease worsening is defined as worsening of PFTs or acute exacerbation of IPF. 1) Worsening of PFTs (confirmed by two tests at least 4 weeks apart) is defined as the occurrence of both: -Decrease from baseline = 10% in FVC (absolute values, i.e., liters) and -Decrease from baseline =15% in DLco (absolute values, i.e., ml×mmHg 1×min-1) A patient unable to perform PFTs at a planned visit due to worsening of IPF will be considered as having a worsening of PFTs if the latter are not invalidated by a test at a follow-up visit. 2) Acute exacerbation of IPF is defined as:An unexplained rapid deterioration of patient’s condition within 4 weeks with an increasing shortness of breath requiring hospitalization and oxygen supplementation = 5 liters/min to maintain a resting SaO2=90% or PaO2 = 55 mmHg (sea level) or 50 mmHg (high altitude). A documented acute exacerbation of IPF will be considered to be an event, irrespective of the results of any follow-up PFTs or fatal outcome. Patients who are transplanted (without a prior documented disease worsening), or who withdraw their consent, or those lost to follow-up before the EOS will be censored at the patient’s EOS visit date. Patients starting forbidden IPF medications (without prior documented disease worsening as defined above) will not be considered as having reached an event. The risk of an event in one group relative to the other is not expected to change with time. The risk of an event in the placebo group is expected to be 25% / year. The treatment effect to be de | — |
Countries
Austria, Belgium, Czech Republic, Denmark, France, Germany, Ireland, Italy, Netherlands, Spain, United Kingdom
Outcome results
None listed