Pulmonary hypertension in patients with interstitial lung disease. Specifically this trial looks at patients who have mild pulmonary hypertension, or are in an at-risk group to developing pulmonary hypertension, in the context of their interstitial lung disease. The interstitial lung diseases addressed include idiopathic pulmonary fibrosis and non-specific interstitial pneumonitis. MedDRA version: 9.1 Level: LLT Classification code 10064911 Term: Pulmonary arterial hypertension
Conditions
Interventions
Trade Name: Tracleer
Product Name: Tracleer
Pharmaceutical Form: Film-coated tablet
Pharmaceutical form of the placebo: Film-coated tablet
Route of administration of the placebo: Oral use
Sponsors
Royal Brompton and Harefield NHS Trust
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1. Patients >=18yrs, =65 years) yes F.1.3.1 Number of subjects for this age range
Exclusion criteria
Exclusion criteria: 1. Patients 80yrs. 2. Patients with unstable disease, or an acute exacerbation of their underlying fibrotic lung disease. 3. Patients with significant other organ co-morbidity including hepatic or renal impairment. 4. Patients with systolic BP < 85mmHg 5. Patients with other conditions that may affect the ability to perform a 6-minute walk test. 6. Patients unable to provide informed consent and comply with the patient protocol. 7. Patients receiving excluded medications (including: epoprostenol, or prostacyclin analogues, phosphodiesterase inhibitors, other endothelin receptor antagonists, drugs with potential interaction with bosentan such as glibenclamide, fluconazole, cyclosporin A, or tacrolimus, and other invesgational agents). 8. Patients with planned surgical intervention during the study period. 9. Pregnant patients 10. Patients with resting oxygen saturation <90% 11. Patients with clinically overt ischaemic heart disease 12. Patients with predominant emphysema on CT scan (in excess of interstitial changes)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main Objective: To efficacy of bosentan in preventing the development of established pulmonary hypertension in patients with fibrosing lung disease considered at-risk of developing pulmonary hypertension. The primary endpoint is the change in resting pulmonary vascular resistance from baseline to week 52. ;Secondary Objective: To assess the ability of bosentan to effect exercise capacity, and pulmonary blood flow, lung function and quality of life in patients with fibrosing lung disease at risk of developing pulmonary arterial hypertension. The secondary endpoints are changes from baseline to week 52 in the followin parameters: 1. Exercise capacity as measured by the 6-minute walk test, and Borg dyspnoea scale 2. Pulmonary blood flow 3. Lung function (DLco% predicted, FVC % predicted, composite physiological index) 4. Right heart catheter data (mean pulmonary arterial pressure, systolic pulmonary arterial pressure, mean right atrial pressure, systemic vascular resistance and cardiac index) 5. Quality of life as measured by teh CAMPHOR questionnaire 6. WHO functional class 7. Time to decline (Death, transplanation, hospitalisation , supplemental oxygen, disease progression). 8. Right ventricular mass (on cardiac MRI) 9. Brain natriuretic peptide ;Primary end point(s): The primary endpoint is the change in resting pulmonary vascular resistance from baseline to week 52 | — |
Countries
United Kingdom
Outcome results
None listed