Sildenafil to Increase Exercise Capacity in Individuals With Idiopathic Pulmonary Fibrosis and Pulmonary Hypertension
Pulmonary Fibrosis, Hypertension, Pulmonary
About this trial
This is an interventional treatment trial for Pulmonary Fibrosis focused on measuring Pulmonary Hypertension, Idiopathic Pulmonary Fibrosis
Eligibility Criteria
Inclusion Criteria: IPF, diagnosed according to the American Thoracic Society (ATS) and European Respiratory Society (ERS) consensus statement (with or without surgical lung biopsy) Pulmonary hypertension, defined as mean pulmonary artery pressure (MPAP) greater than or equal to 25 mm Hg by right heart catheterization (RHC) Exclusion Criteria: Interstitial lung disease due to conditions other than IPF Recent lung or upper respiratory tract infection within 4 weeks of study entry Acute or chronic impairment other than dyspnea (e.g., angina pectoris, intermittent claudication) limiting the ability to comply with study requirements (e.g., 6-minute walk test) Known hypersensitivity to sildenafil Known or suspected coronary artery disease (CAD) Unstable angina Nitrate use Known or suspected aortic stenosis (AS) Known or suspected heart attack, stroke, or life-threatening arrythmias within 1 month of study entry Severe chronic heart failure, defined as New York Heart Association (NYHA) class III/IV and/or left ventricular ejection fraction less than 25% Known penile deformities Known kidney or liver dysfunction Uncontrolled diabetes (blood glucose less than 60 mg/dl or greater than 300 mg/dl) Severe serum sodium abnormalities (serum sodium less than 130 mEq/L or greater than 150 mEq/L) Condition that may predispose participant to priapism (e.g., sickle cell anemia, multiple myeloma, leukemia) Retinitis pigmentosa Known or suspected idiopathic hypertrophic subaortic stenosis (IHSS) Low blood pressure (systolic blood pressure [SBP] less than 100 mm Hg or diastolic blood pressure [DBP] less than 50 mm Hg) Uncontrolled systemic high blood pressure (SBP greater than 180 mm Hg or DBP greater than 100 mm Hg) Cardiopulmonary rehabilitation program started within 8 weeks of study entry or likely to start prior to the conclusion of the study Treatment with an endothelin receptor antagonist, iloprost, epoprostenol, inhibitors of CYP3A4 (e.g., cimetidine, erythromycin, ketoconazole, itraconazole, mibefradil), protease inhibitors (e.g., amprenavir, indinavir, or ritonavir), rifampin, alpha-blockers (e.g., doxazosin), or other phosphodiesterase-5 inhibitors Current use of alcohol, grapefruit juice, or St. John's wort Pregnant or breastfeeding
Sites / Locations
- UCLA Pulmonary Outpatient Clinic,