At each study visit after Visit one, the following tests will be completed. * Vital signs (blood pressure, heart rate) and weight measured. *Electrocardiogram (ECG, a tracing of the electrical activity of your heart) *Echocardiogram (ECHO, an ultrasound of the heart) will be done as well. *6 minute walk test *You will have blood drawn for laboratory testing (approximately 5 teaspoons). At the baseline visit and the year 3 visit only a right heart catheterization will be performed.

3

There are two purposes for this research study. The first purpose is to try to evaluate different non-invasive screening methods to see how well they can predict and confirm PAH/PH in scleroderma subjects. Currently, right heart catheterization is the standard test to diagnose PAH/PH. It is invasive and can be costly. The second purpose of this study is to investigate how many scleroderma subjects develop PAH/PH.

To participate in the study, there are certain requirements that must be met. At Visit 1, your study doctor will decide if you qualify for the study. If you are included in the study, you will have certain medical procedures and tests done at a yearly visit over a total of 3 years. At each study visit after Visit one, the following tests will be completed. * Vital signs (blood pressure, heart rate) and weight measured. *Electrocardiogram (ECG, a tracing of the electrical activity of your heart) *Echocardiogram (ECHO, an ultrasound of the heart) will be done as well. *6 minute walk test *You will have blood drawn for laboratory testing (approximately 5 teaspoons). At the baseline visit and the year 3 visit only a right heart catheterization will be performed. A catheter (narrow tube) will be inserted into your right heart. This will be used to determine certain parameters that measure the blood flow and blood pressures in your heart and lung. Right heart catheterization is performed by inserting a small catheter in a vein of the neck, arm or groin, under local anesthesia. Using a TV screen and x-rays, the study doctor will then thread the catheter along the vein, through the heart, and into the blood vessels going to your lungs. Right heart catheterization is the current gold standard confirmatory test for PAH/PH.