Tadalafil for Sarcoidosis Associated Pulmonary Hypertension

Inclusion Criteria:

- Biopsy proven sarcoidosis

- Mean pulmonary artery pressure > 25 mmHg at rest or 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% predicted

- Forced expiratory volume in 1 second (FEV1) > 40% predicted

- 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, vardenafil, treprostinil,
epoprostenol, iloprost, bosentan, ambrisentan) 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

- Severe systemic hypotension < 90/50

- History of priapism

- Patients with congestive heart failure (left ventricular dysfunction) LVEF < 45% by
echocardiogram

- 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, chronic thromboemboli)

- Use within 1 month of an sildenafil or vardenafil

- WHO functional class IV status

- Patients with severe other organ disease felt by investigators to impact on survival
during the course of the study

- Subjects with liver function abnormalities (ALT or AST > 3 times the upper limit of
normal at screening or at baseline) or chronic liver disease

- Advanced kidney failure (GFR < 30 ml/min at screening or at baseline)

- History of hypersensitivity reaction or adverse effect related to tadalafil

- Pregnant or lactating women

- Concomitant use of nitrates (any form) either regularly or intermittently

- Concomitant use of potent CYP3A inhibitors (eg, ritonavir, ketoconazole,
itraconazole)

- Any additional contraindications and precautions specified in the package inserts for
Tadalafil (Adcirca) not listed above