Pulmonary arterial hypertension (PAH) is a condition characterized by an increased pulmonary
vascular resistance that can lead to right heart failure and death. Several diseases are
known etiologies of PAH including scleroderma and cirrhosis. The presence of PAH in the
context of systemic sclerosis or cirrhosis has a dramatic impact on prognosis and survival of
the connective tissue or liver disease.
Despite advances in the diagnosis of PAH, echocardiography remains a necessary test for
screening PAH in patients with scleroderma or cirrhosis. However, echocardiography is less
than ideal for diagnosing PAH and predicting treatment response. Thus, there is a pressing
need to identify methodologies that can accurately and non-invasively recognize the presence
of PAH in patients with scleroderma and cirrhosis.
1. To measure endothelial function and exhaled gases in patients with scleroderma and
cirrhosis. To assess whether they correlate with the presence or the development of PAH.
2. The degree of local (forearm) capillary vasodilation during treprostinil iontophoresis
identifies patients who will develop PAH and in those already diagnosed PAH predicts
response to PAH-specific therapies.