Endothelial Function in Patients With Scleroderma or Cirrhosis With and Without Pulmonary Hypertension

Study Overview
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Brief Summary
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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: 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. 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.

Detailed Description
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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: 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. 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.

Official Title
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Endothelial Function in Patients With Scleroderma or Cirrhosis With and Without Pulmonary Hypertension

Conditions
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Pulmonary Hypertension

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients with scleroderma or cirrhosis. Exclusion Criteria: Exclusion criteria include individuals younger than 16, etiologies of PAH other than scleroderma or cirrhosis.

Ages Eligible for Study
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Minimum Age: 17 Years
Maximum Age: 90 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Scleroderma<br>60 patients with scleroderma - 30 with and 30 without Pulmonary Arterial Hypertension | |
| Cirrhosis<br>60 patients with cirrhosis - 30 with and 30 without Pulmonary Arteria Hypertension | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Base Line | Baseline characteristics in patients with and without PAH will be compared using parametric (t-test) and non parametric (Mann-Whitney) tests for continuous data and Chi-square for categorical data. | up to 3 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Scleroderma, Cirrhosis