Inclusion Criteria:

• Male and female patients, age ≥ 18 years at time of signing informed written consent.

Exclusion Criteria:

• Clinically significant pulmonary hypertension, based on any of the following criteria:
  • Receiving treatment for pulmonary arterial hypertension; or
  • Evidence for any form of pulmonary hypertension as determined by previous right heart catheterization. Pulmonary arterial hypertension is defined as a mean pulmonary artery pressure ≥ 20 mmHg at rest, a mean pulmonary capillary wedge pressure ≤ 15 mmHg, and the pulmonary vascular resistance ≥ 3 Wood units; or
  • Doppler echocardiography within the 6 months before study Day 1 reporting an estimated right ventricular systolic pressure of >40 mm Hg if subsequent confirmatory right heart catheterization has not been done.
• Doppler echocardiography in the 6 months before study Day 1 showing evidence of right atrial or right ventricular enlargement or significant left ventricular dysfunction with left ventricular ejection fraction < 40%.
• Clinical evidence of significant aspiration or uncontrolled gastroesophageal reflux (i.e., University of California, Los Angeles, Scleroderma Clinical Trial Consortium Gastrointestinal Scale, 2.0 [UCLA SCTC GIT 2.0] reflux score >1.00).
• Known esophageal dysfunction with esophageal stricture sufficient to limit the ability to swallow oral medication.
• Prior history of renal crisis.