Selected

Inclusion Criteria:

- Diagnosis of IPF based on modified American Thoracic Society-European respiratory
Society (ATS-ERS) guidelines

- Diagnosis of PH based on the following hemodynamic requirements: mean pulmonary
artery pressure (mPAP_ ≥25 mmHg; pulmonary vascular resistance (PVR) >240
dyne.sec/cm5; pulmonary capillary wedge pressure (PCWP) or left ventricular
end-diastolic pressure (LVEDP) ≤15 mmHg

- Forced vital capacity (FVC) ≥40%

- Able to walk at least 50 meters during two 6 minute walk tests

- If receiving calcium channel blockers, low dose oral corticosteroids,
immunosuppressive, cytoxic, or antifibrotic drugs dose must be stable.

Selected Exclusion Criteria:

- Diagnosis of PH primarily due to an etiology other than IPF

- Surgical lung biopsy diagnosis other than Usual Interstitial Pneumonia

- Other known cause of interstitial lung disease

- Evidence of significant obstructive lung disease

- Recent hospitalization for an acute exacerbation of IPF

- Recent active pulmonary or upper respiratory tract infection

- Left ventricular ejection fraction (LVEF) <40%

- Serum creatinine ≥2.5 mg/dL

- Requires hemodialysis, peritoneal dialysis or hemofiltration

- Female subject who is pregnant or breastfeeding

- Recent treatment for PH with an ERA, phosphodiesterase type 5 (PDE-5) inhibitor, or
prostacyclin derivative

- Recent treatment with high dose oral corticosteroids

- Recent treatment (within 4 weeks prior to screening)with imatinib mesylate (Gleevec)

- Alanine aminotransferase (ALT) or aspartate aminotransferase (AST) lab value that is
greater than 1.5x the upper limit of the normal range (ULN)

- Discontinued other ERA treatment for any adverse reaction other than those associated
with liver function test abnormalities