Novel Screening Strategies for Scleroderma PAH

Novel Screening Strategies for Scleroderma PAH (Pulmonary Arterial Hypertension)

Patients with scleroderma can develop heart failure due to high blood pressure in the lungs (a condition called pulmonary arterial hypertension). It is important to find pulmonary arterial hypertension early, so that it can be treated before heart failure develops. However, the tests that we now use to find the earliest form of this disease in scleroderma patients are not good enough. This study will examine whether tests performed during exercise can improve our ability to find early pulmonary arterial hypertension. The study will also try to identify genes that are responsible for the development of pulmonary arterial hypertension.

Study Overview

• Status: Completed
• Conditions: Pulmonary Arterial Hypertension; Scleroderma

• Study Type: Observational
• Enrollment (Actual): 156

Contacts and Locations

Study Locations

• United States
  • Michigan
    • Ann Arbor, Michigan, United States, 48109
      • University of Michigan

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 30 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Volunteers will be recruited from scleroderma clinics, pulmonary hypertension clinics, and (for healthy volunteers) the Ann Arbor area.

Description

Inclusion Criteria:

• 30 years or older;
• diagnosis of limited or diffuse scleroderma (American College of Rheumatology criteria)

• for the "high risk" group, one of the following features:

  • resting transthoracic echocardiogram showing elevated right-sided pressures within previous 3 months [tricuspid regurgitation (TR) jet >2.8 m/s or evidence of right ventricular dysfunction]
  • pulmonary function testing (PFT) showing abnormal diffusing capacity of carbon monoxide (DLCO) not due to significant interstitial lung disease (DLCO<60% predicted or FVC: DLCO ratio >1.4)

Exclusion Criteria:

• Pregnancy
• prior diagnosis of pulmonary hypertension
• treatment with endothelin receptor antagonists, phosphodiesterase-5 inhibitors, or prostacyclin analogues
• previous diagnosis of obstructive lung disease or pulmonary thromboembolic disease
• current smoker
• significant valvular disease
• resting echocardiogram showing left ventricular ejection fraction<50% within previous 3 months
• resting echocardiogram showing significant (greater than Grade I) diastolic dysfunction
• pulmonary emboli (past or present).

Study Plan

How is the study designed?

Number of groups / cohorts

4

Cohorts and Interventions

Group / Cohort
Healthy volunteers
Scleroderma and diagnosed PAH
"Low risk" scleroderma
"High risk" scleroderma

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Development of pulmonary arterial hypertension	Two years after enrollment.

Collaborators and Investigators

• Sponsor: University of Michigan
• Investigators: Principal Investigator: Scott H Visovatti, MD, University of Michigan

Study record dates

Study Major Dates

• Study Start (Actual): September 26, 2013
• Primary Completion (Actual): November 6, 2019
• Study Completion (Actual): November 6, 2019

Study Registration Dates

• First Submitted: October 8, 2013
• First Submitted That Met QC Criteria: October 9, 2013
• First Posted (Estimate): October 10, 2013

Study Record Updates

• Last Update Posted (Actual): November 15, 2021
• Last Update Submitted That Met QC Criteria: November 10, 2021
• Last Verified: November 1, 2021

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Skin Diseases; Respiratory Tract Diseases; Lung Diseases; Connective Tissue Diseases; Hypertension, Pulmonary; Hypertension; Scleroderma, Systemic; Scleroderma, Diffuse; Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension; Scleroderma, Localized
• Other Study ID Numbers: HUM00074818