Patient case (clinical description): A 47-year-old man comes to the office for routine checkup. He is complaining of chronic cough and occasional but progressive dyspnea.  Other medical conditions include hypertension and osteoarthritis.  The patient smokes a pack of cigarettes daily and does not use alcohol or illicit drugs.  He used to be a construction worker. On examination, there are decreased breath sounds and percussive dullness at the base of both lungs.  Chest CT scan reveals a mild bilateral pleural effusion and diffuse thickening of the pleura. The patient's documents show chronic exposure to asbestosis. The specimen of the lungs reveled  pulmonary fibrosis that is most predominant in the lower lobes, characterized  by the presence of asbestos bodies (golden-brown beaded rods with translucent centers).

Patient's own account: I'm a 47-year-old former construction worker. I'm a man and I smoke a pack of cigs a day but I don't drink or do drugs. I went to my routine checkup because I've been coughing so much lately. I also had shortness of breath and it got worse over time. I already suffer from hypertension and osteoarthritis. My doctor found that I have decreased breath sounds. I did a chest scan and it seems that I have fluid around my lungs, and the lining of my lungs has become thicker in many areas. I have been exposed a lot to asbestos fibers. The other lung exam showed that I have pulmonary fibrosis, especially in the lower part of my lungs and they found some asbestos there.

Clinical trial: Sildenafil to Increase Exercise Capacity in Individuals With Idiopathic Pulmonary Fibrosis and Pulmonary Hypertension

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
No condition on gender to be admitted to the trial.
No healthy subjects accepted to join the trial.
Subject must be at least 19 Years old.


Eligibility (NLI label): Entailment