Endothelial Function in Patients With Scleroderma or Cirrhosis With and Without Pulmonary Hypertension
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|ClinicalTrials.gov Identifier: NCT01729611|
Recruitment Status : Recruiting
First Posted : November 20, 2012
Last Update Posted : April 12, 2017
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.
- 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.
|Condition or disease|
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.
- 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.
|Study Type :||Observational|
|Estimated Enrollment :||140 participants|
|Official Title:||Endothelial Function in Patients With Scleroderma or Cirrhosis With and Without Pulmonary Hypertension|
|Study Start Date :||December 2013|
|Estimated Primary Completion Date :||March 2018|
|Estimated Study Completion Date :||March 2018|
60 patients with scleroderma - 30 with and 30 without Pulmonary Arterial Hypertension
60 patients with cirrhosis - 30 with and 30 without Pulmonary Arteria Hypertension
- Base Line [ Time Frame: up to 3 months ]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.
To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT01729611
|Contact: Adriano Tonelli, MDfirstname.lastname@example.org|
|United States, Ohio|
|Cleveland, Ohio, United States, 44195|
|Principal Investigator:||Adriano Tonelli, MD||The Cleveland Clinic|