Bosentan in Pulmonary Hypertension in Interstitial Lung Disease Treatment Study

Launched by ROYAL BROMPTON & HAREFIELD NHS FOUNDATION TRUST · Mar 10, 2008

Keywords

ClinConnect Summary

• Purpose: High blood pressure in the lungs or pulmonary hypertension (PH) is a common complication of fibrosing (or interstitial, ILD) lung disease. When present, it is associated with markedly reduced prognosis and survival. Endothelin-1 (ET-1)is over-expressed in patients with PH and ILD, and is thought to play a role in the development of both conditions. Bosentan blocks the action of ET-1, and has been shown to be beneficial in patients with PH from an unknown cause, or related to other conditions (such as heart conditions, connective-tissue disease, and HIV). It is important to establ...

Gender

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Eligibility 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).