One hundred fifty-five patients with IPF were identified in the database. A substantial percentage of these patients (47 of 117 patients, 40.1%) had PH based on echocardiography (estimated right ventricular systolic pressure > 30 mm Hg). Interestingly, 20 of 117 patients (17.1%) had moderate-to-severe PH (estimated right ventricular systolic pressure > 45 mm Hg). We identified 88 probes on the Uniset arrays that represent 86 unique genes known to be associated with vascular endothelium. The majority (69.8%) of these genes were unchanged in IPF. Surprisingly, underexpressed genes (19.8%) included angiogenic factors such as vascular endothelial growth factor and platelet endothelial cell adhesion molecule, as well as factors affecting vessel tone such as angiotensin-converting enzyme and endothelin-1 (p value < 0.05). Overexpressed genes included phospholipase A2 (10.4%) and other factors that mediate remodeling.