Endothelial Function in Patients With Scleroderma or Cirrhosis With and Without Pulmonary Hypertension
| Status: | Recruiting |
|---|---|
| Conditions: | High Blood Pressure (Hypertension), Gastrointestinal, Dermatology |
| Therapuetic Areas: | Cardiology / Vascular Diseases, Dermatology / Plastic Surgery, Gastroenterology |
| Healthy: | No |
| Age Range: | 17 - 90 |
| Updated: | 3/6/2019 |
| Start Date: | December 2013 |
| End Date: | July 2020 |
| Contact: | Adriano Tonelli, MD |
| Email: | tonella@ccf.org |
| Phone: | 216.444.0812 |
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.
Hypothesis:
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.
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.
Hypothesis:
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.
Patients with scleroderma are known to have endothelial dysfunction and limited data
suggested an association between the degree of endothelial function in scleroderma and the
presence of PAH. However, these data is preliminary and has not been used to predict response
to PAH-specific therapy or the development of PAH. We will test patients with cirrhosis
because they tend to have PAH in the context of a hyperdynamic instead of a hypodynamic state
as observed in scleroderma and PAH.
Aims:
1. To measure endothelial function and exhaled gases in patients with scleroderma or
cirrhosis to assess whether they correlate with the presence or the development of PAH.
2. To evaluate the degree of endothelial response to local treprostinil iontophoresis and
determine if this test can predict the development of pulmonary hypertension or response
to PAH-specific therapies.
suggested an association between the degree of endothelial function in scleroderma and the
presence of PAH. However, these data is preliminary and has not been used to predict response
to PAH-specific therapy or the development of PAH. We will test patients with cirrhosis
because they tend to have PAH in the context of a hyperdynamic instead of a hypodynamic state
as observed in scleroderma and PAH.
Aims:
1. To measure endothelial function and exhaled gases in patients with scleroderma or
cirrhosis to assess whether they correlate with the presence or the development of PAH.
2. To evaluate the degree of endothelial response to local treprostinil iontophoresis and
determine if this test can predict the development of pulmonary hypertension or response
to PAH-specific therapies.
Inclusion Criteria:
- Patients with scleroderma or cirrhosis.
Exclusion Criteria:
- Exclusion criteria include individuals younger than 16, etiologies of PAH other than
scleroderma or cirrhosis.
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