Bosentan in Pulmonary Hypertension in Interstitial Lung Disease Treatment Study

• Status: Unknown status
• Trial end date: 2010-08-01
• Target enrollment: 0
• Participant gender: All

Summary

Over time, patients with fibrosing or interstitial lung disease (ILD) can develop high lung blood pressures (pulmonary hypertension), and this is associated with poorer prognosis and survival. It is thought that development of PH contributes to the deterioration and death of patients with ILD. Endothelin-1 (ET1) is a substance contributing to the development of both PH and ILD. Bosentan is a drug blocking the action of ET-1 by binding to its receptors. Bosentan clearly benefits patients with PH of unknown cause, or related to other diseases (such as heart conditions, or HIV) both alone and in combination with other treatments. In patients with fibrosing lung disease and PH, there have been no controlled treatment studies. Clearly it is important to evaluate the effectiveness of bosentan in these patients.

This study aims to determine the ability of bosentan to reduce high blood pressure in the lungs (pulmonary hypertension) in patients with scarring (fibrosing) lung disease. It is a placebo-controlled double blinded study for 16 weeks (and it is proposed to follow patients in a 16 week open-label phase with bosentan therapy).

• Phase: Phase 4
• Accepts Healthy Volunteers?: No

Details

• Lead Sponsor: Royal Brompton & Harefield NHS Foundation Trust
• Collaborator: Actelion
• Treatments: Bosentan; Endothelin Receptor Antagonists

Criteria

Inclusion Criteria:

1. Patients >=18yrs, <80yrs

2. Patients with idiopathic pulmonary fibrosis (IPF) or idiopathic fibrotic non-specific interstitial pneumonitis (NSIP) confirmed by their respiratory physician according to ATS/ERS criteria.

3. Patients with pulmonary hypertension on right heart catheter (mean pulmonary arterial pressure >=25mmHg with pulmonary artery occlusion pressure, left atrial pressure or left ventricular end-diastolic pressure <15mmHg).

4. Patients providing written informed consent.

Exclusion Criteria:

1. Patients <18, >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 investigational agents).

8. Patients with planned surgical intervention during the study period.

9. Pregnant patients or women of child-bearing age, who are not using a reliable contraceptive method.

10. Patients with clinically overt ischaemic heart disease.

11. Patients with predominant emphysema on high resolution CT scan (emphysema greater in extent than interstitial changes).