Pulmonary Hypertension, Scleroderma, Systemic Sclerosis
Conditions
Brief summary
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.
Interventions
There is no intervention for this study
Sponsors
University of South Alabama
Louisiana State University Health Sciences Center in New Orleans
Study design
Observational model
COHORT
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
Yes
Inclusion criteria
For systemic sclerosis subjects: Inclusion Criteria: * Age \>18 years * Meet American College of Rheumatology criteria for systemic sclerosis
Exclusion criteria
* Chronic kidney disease (estimated creatinine clearance \<50mL/min) * Uncontrolled hypertension (diastolic blood pressure\>120mmHg) * Acute coronary syndrome within the past 6 months * Chronic obstructive pulmonary disease * Diabetes mellitus * Hemolytic anemia * Active tobacco abuse For healthy control subjects: Inclusion Criteria: * Age\>18 years * Age- and sex-matched to SSc patients
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Plasma VE-cadherin + endothelial microparticle levels | Baseline | endothelial microparticles are expressed as microparticles per microliter of plasma. This is a cross-sectional analysis, so the measurements will be performed once, on study visit 1 |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Plasma PECAM+ endothelial microparticles | Baseline | endothelial microparticles are expressed as microparticles per microliter of plasma. This is a cross-sectional analysis, so the measurements will be performed once, on study visit 1 |
| Plasma E-selectin + endothelial microparticles | Baseline | endothelial microparticles are expressed as microparticles per microliter of plasma. This is a cross-sectional analysis, so the measurements will be performed once, on study visit 1 |
Countries
United States
Outcome results
None listed