Ambrisentan (Letairis) for Sarcoidosis Associated Pulmonary Hypertension

Primary Endpoint: Change in 6 minute walk distance.

Inclusion Criteria:

- Biopsy proven sarcoidosis

- Mean pulmonary artery pressure > 25 mmHg at rest and greater than 30 mmHg with
exercise by right heart catheterization within 1 year prior to entry into study

- Pulmonary capillary wedge pressure ≤ 15 mmHg

- PVR values >3.0 Woods units

- Forced vital capacity (FVC) >40%

- WHO functional class II or III

- Stable sarcoidosis treatment regimen for three months prior to entry into study

- 6 minute walk distance between 150-450 meters

- Stable dose of antihypertensive medications

- On no other medication to treat PAH (sildenafil, tadalafil, vardenafil, treprostinil,
epoprostenol, iloprost, bosentan, sitaxsentan) within one month prior to enrollment
and during duration of the study

- Non-pregnant females

Exclusion Criteria:

- Exercise limitation related to a non-cardiopulmonary reason (e.g. arthritis)

- Severe systemic hypertension > 170/95

- Patients with congestive heart failure (left ventricular dysfunction) or primary right
ventricular dysfunction

- Anticipation by the investigator for escalation in sarcoidosis treatment during the
course of the study

- Pulmonary hypertension related to etiology other than sarcoidosis (i.e. HIV,
scleroderma, etc.)

- Use within 1 month of an endothelin receptor antagonists (bosentan, sitaxsentan).

- WHO functional class IV status

- Patients with significant left ventricular dysfunction

- 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.