Double Blind, Randomized Trial of Bosentan for Sarcoidosis Associated Pulmonary Hypertension

Patients will be randomized in 2:1 manner to receive either bosentan or placebo for 16 weeks. After 16 weeks, there will be an additional 32 weeks of an open label extension.

Inclusion criteria

• Patients with known sarcoidosis 21.
• Age 18 or greater
• Patients with documented pulmonary hypertension with a PA mean > 25 mm Hg as measured by cardiac catheterization within six months of entry into the study. Pulmonary artery occluding pressure and or left ventricular end diastolic pressure must be less than 15 mm Hg.
• Patients with WHO class II or III
• Six minute walk distance of between 100 to 500 meters
• Patients on stable immunotherapy for their sarcoidosis, including prednisone, methotrexate, azathioprine, hydroxychloroquine, cyclophosphamide, thalidomide, and/or infliximab
• Patients able to provide written consent

Exclusion criteria

• Patients on pulmonary vasodilator drugs (flolan, remodulin, bosentan, sildenafil) n the prior 28 days. Patients on stable dose of calcium channel blocker for more than 1 month prior to right heart catheterization can be continued on the calcium channel blocker.
• Patients with severe airway obstruction as defined by FEV1/FVC of less than 35%
• Patients with World Health Organization (WHO) class IV status.
• Patients who are pregnant or breast feeding
• Patients with significant left ventricular dysfunction with a left ventricular ejection fraction of less than 35%
• Cardiac index < 2.0 liters and/or right atrial pressure >15 mm Hg
• Significant liver dysfunction not due to sarcoidosis.
• Patients with severe other organ disease felt by investigators to impact on survival during the course of the study.
• Patients unable to perform the 6 minute walk study