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

NCT01729611 · Status: COMPLETED · Type: OBSERVATIONAL · Enrollment: 119

Last updated 2021-04-02

No results posted yet for this study

Summary

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.

Conditions

  • Pulmonary Hypertension

Sponsors & Collaborators

  • The Cleveland Clinic

    lead OTHER

Principal Investigators

  • Adriano Tonelli, MD · The Cleveland Clinic

Eligibility

Min Age
17 Years
Max Age
90 Years
Sex
ALL
Healthy Volunteers
No

Timeline & Regulatory

Start
2013-12-31
Primary Completion
2021-03-31
Completion
2021-04-30

Countries

  • United States

Study Locations

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Read the full study record

This page highlights key information. For complete eligibility criteria, study locations, investigator contacts, and the full protocol, visit the original record on ClinicalTrials.gov.

View NCT01729611 on ClinicalTrials.gov