Novel Screening Strategies for Scleroderma PAH

NCT01959815 | Completed DMC

• 1 other identifier: HUM00074818
• Study Type: observational
• Target: 156
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

Pulmonary Arterial Hypertension; Scleroderma

Outcome Measures

Primary Outcomes (1)

• Development of pulmonary arterial hypertension

  Two years after enrollment.

Study Arms (4)

Scleroderma and diagnosed PAH

"Low risk" scleroderma

Healthy volunteers

"High risk" scleroderma

Eligibility Criteria

• Age: 30 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

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

You may qualify if:

• 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)

You may not qualify if:

• 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).

Sponsors & Collaborators

• University of Michigan: lead

Study Sites (1)

University of Michigan

Ann Arbor, Michigan, 48109, United States

Location

Biospecimen

Blood samples will be drawn at rest and during exercise.

MeSH Terms

Conditions

Pulmonary Arterial Hypertension; Scleroderma, Diffuse

Condition Hierarchy (Ancestors)

Hypertension, Pulmonary; Lung Diseases; Respiratory Tract Diseases; Scleroderma, Systemic; Connective Tissue Diseases; Skin and Connective Tissue Diseases; Skin Diseases

Study Officials

• Scott H Visovatti, MD

  University of Michigan

  PRINCIPAL INVESTIGATOR

Study Design

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

Study Record Dates

• First Submitted: October 8, 2013
• First Posted: October 10, 2013
• Study Start: September 26, 2013
• Primary Completion: November 6, 2019
• Study Completion: November 6, 2019
• Last Updated: November 15, 2021

Record last verified: 2021-11

Locations

US (1)