Novel Screening Strategies for Scleroderma PAH

University of Michigan

Status: Completed

Conditions: Scleroderma; Pulmonary Arterial Hypertension

Study type: Observational

Funder types: Other

Identifiers

NCT01959815

HUM00074818

Details and patient eligibility

About

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.

Enrollment: 156 patients

Sex: All

Ages: 30+ years old

Volunteers: Accepts Healthy Volunteers

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).

Trial design

156 participants in 4 patient groups

Scleroderma and diagnosed PAH

"Low risk" scleroderma

Healthy volunteers

"High risk" scleroderma