Recruitment

Recruitment Status
Recruiting

Inclusion Criterias

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

Exclusion Criterias

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

Summary

Conditions
  • Pulmonary Arterial Hypertension
  • Scleroderma
Type
Observational
Design
  • Observational Model: Cohort
  • Time Perspective: Prospective

Participation Requirements

Age
Between 30 years and 125 years
Gender
Both males and females

Inclusion Criterias

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

Exclusion Criterias

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

Locations

Ann Arbor, Michigan, 48109
Ann Arbor, Michigan, 48109

Tracking Information

NCT #
NCT01959815
Collaborators
Not Provided
Investigators
Not Provided