Novel Screening Strategies for Scleroderma PAH

Sponsor

University of Michigan (Other)

Overall Status

Completed

CT.gov ID

NCT01959815

Collaborator

(none)

156

Enrollment

Location

73.3

Actual Duration (Months)

2.1

Patients Per Site Per Month

Study Details

Study Description

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.

Condition or Disease	Intervention/Treatment	Phase
Pulmonary Arterial Hypertension Scleroderma

Study Design

Study Type:

Observational

Actual Enrollment :

156 participants

Observational Model:

Cohort

Time Perspective:

Prospective

Official Title:

Novel Screening Strategies for Scleroderma PAH (Pulmonary Arterial Hypertension)

Actual Study Start Date :

Sep 26, 2013

Actual Primary Completion Date :

Nov 6, 2019

Actual Study Completion Date :

Nov 6, 2019

Arms and Interventions

Arm	Intervention/Treatment
Scleroderma and diagnosed PAH
"Low risk" scleroderma
Healthy volunteers
"High risk" scleroderma

Outcome Measures

Primary Outcome Measures

Development of pulmonary arterial hypertension [Two years after enrollment.]

Eligibility Criteria

Criteria

Ages Eligible for Study:

30 Years and Older

Sexes Eligible for Study:

All

Accepts Healthy Volunteers:

Yes

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).