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CompletedNCT01729611Updated Apr 2, 2021

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

An observational study in Pulmonary Hypertension, sponsored by The Cleveland Clinic. Completed at 1 site in United States. Open to participants aged 17 Years to 90 Years. Per ClinicalTrials.gov, last updated 2021-04-02.

Sponsored by The Cleveland Clinic · Observational

Study type
Observational
Model
Cohort
Time perspective
Prospective
Enrollment
119
Ages
17 Years to 90 Years
Sex
All
01

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.
Read the detailed description

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.
02

Conditions studied

03

In context

Hypertension, Pulmonary

1,105 studies on the registry are indexed under Hypertension, Pulmonary; 234 are open to participants now.

This study's enrollment of 119 is close to the median of 116 across 386 observational studies indexed under Hypertension, Pulmonary.

Browse Hypertension, Pulmonary studies →

Lead sponsor

The Cleveland Clinic is the lead sponsor of 818 studies on the registry; 118 are open to participants now.

Of its 89 completed or terminated interventional studies of FDA-regulated products, 72 (81%) have results posted.

Counted across the registry records on this site, refreshed daily.

04

Who can participate

Ages eligible
17 Years to 90 Years
Sexes eligible
All
Accepts healthy volunteers
No
Sampling method
Non-probability sample

Study population

Patients with scleroderma or cirrhosis. These patients may or may not have known PAH or be on treatment of this condition.

Inclusion criteria

  • Patients with scleroderma or cirrhosis.

Exclusion criteria

Exclusion Criteria:

  • Exclusion criteria include individuals younger than 16, etiologies of PAH other than scleroderma or cirrhosis.
05

Study design

Observational model
Cohort
Time perspective
Prospective
Enrollment
119 participants (actual)

Groups and cohorts

  • Scleroderma

    60 patients with scleroderma - 30 with and 30 without Pulmonary Arterial Hypertension

  • Cirrhosis

    60 patients with cirrhosis - 30 with and 30 without Pulmonary Arteria Hypertension

06

What researchers measure

Primary outcomes

  1. 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.

    Time frame: up to 3 months

07

Study locations

1 site
  • Cleveland Clinic
    Cleveland, Ohio 44195, United States
08

Updates

Tracking since Sep 25, 2026
No changes since tracking began. The registry record was last updated on Apr 2, 2021, before this site started recording changes on Sep 25, 2026. Its history is on ClinicalTrials.gov ↗
09

Registry details

Key details

Study ID
NCT01729611
Lead sponsor
The Cleveland Clinic
Responsible party
Adriano Tonelli (Staff Physician, The Cleveland Clinic) — Principal investigator
First posted
Nov 20, 2012
Start date
Dec 2013
Primary completion
Mar 2021
Completion
Apr 2021
Last update
Apr 2, 2021

Study contacts

Adriano Tonelli, MD
principal investigator · The Cleveland Clinic

Oversight

Data monitoring committee
No
View the source record on ClinicalTrials.gov ↗

Not currently enrolling

This study is completed, as verified in Apr 2021. You cannot join it, but the record below documents what was studied.

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