Bosentan in Pulmonary Hypertension in Interstitial Lung Disease Treatment Study
Status:
Unknown status
Trial end date:
2010-08-01
Target enrollment:
Participant gender:
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).