Endothelial Microparticles in Systemic Sclerosis Pulmonary Hypertension

Systemic sclerosis (SSc, also known as scleroderma) is a disease characterized by fibrosis
of the skin and organs, inflammation, and an abnormal endothelial cell lining inside of
vessels. A common and deadly complication of SSc is pulmonary hypertension (PH), which is an
abnormal elevation in the blood pressure within the lung blood vessels. Early identification
and treatment of PH is important in SSc, and no clinical factors can predict which patients
will develop PH with acceptable accuracy. A potential marker of PH in SSc is the presence of
increased amounts of endothelial microparticles (EMPs), which are substances circulating in
the blood that were released from damaged vessel wall endothelial lining. A main goal of
this study is to investigate if there is a difference in EMP levels between SSc patients
with and without PH. The investigators will also use human endothelial cells in a lab
environment to test whether these EMPs isolated from SSc patients are actually causing
damage to the vessel lining. Lastly, the investigators will investigate the potential
benefit of a medication used after transplant, mycophenolate mofetil (MMF). This will be
done by causing damage to isolated human endothelial cells and treating them with MMF. The
main goal of this portion of our study is to see if EMP levels are reduced when cells are
treated with MMF. Overall, the investigators anticipate the following outcomes of this
study: 1) use EMP levels to differentiation patients with SSc who have PH from those without
PH, 2) use EMPs to understand how endothelial damage occurs in SSc, and 3) use EMPs to help
us develop new treatments for patients with vascular diseases.