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

NCT01729611 | Completed

• 1 other identifier: Tonella1
• Study Type: observational
• Target: 119
• Locations: 1 country
• Sites: 1

Brief 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. 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. 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

Keywords

Scleroderma; Cirrhosis

Outcome Measures

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.

  up to 3 months

Study Arms (2)

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

Eligibility Criteria

• Age: 17 Years - 90 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• 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.

You may qualify if:

• Patients with scleroderma or cirrhosis.

Sponsors & Collaborators

• The Cleveland Clinic: lead

Study Sites (1)

Cleveland Clinic

Cleveland, Ohio, 44195, United States

Location

MeSH Terms

Conditions

Hypertension, Pulmonary; Scleroderma, Diffuse; Fibrosis

Condition Hierarchy (Ancestors)

Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases; Cardiovascular Diseases; Scleroderma, Systemic; Connective Tissue Diseases; Skin and Connective Tissue Diseases; Skin Diseases; Pathologic Processes; Pathological Conditions, Signs and Symptoms

Study Officials

• Adriano Tonelli, MD

  The Cleveland Clinic

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Staff Physician

Study Record Dates

• First Submitted: November 15, 2012
• First Posted: November 20, 2012
• Study Start: December 1, 2013
• Primary Completion: March 1, 2021
• Study Completion: April 1, 2021
• Last Updated: April 2, 2021

Record last verified: 2021-04

Locations

US (1)