Patients with systemic sclerosis (SSc) related pulmonary arterial hypertension (SScPAH) have a worse prognosis than those with idiopathic PAH. We have recently discovered that heart cells in SScPAH do not contract or squeeze as well as in other forms of pulmonary hypertension. However, the mechanism leading to this dysfunction is not understood. To better study this and in hopes of developing a future therapy, we plan to collect tissue samples via a heart biopsy at the time of a clinically indicated heart catheterization.
The purpose of the study is to generate a bio bank of specimens for research. We will tissue that would otherwise be discarded from clinical or surgical procedure and information from medical records. We will also collect discarded blood, urines and sputum. Collecting samples will help to better understanding the mechanisms of cardiovascular diseases, identify biomarkers for early diagnosis and to predict safety and efficacy of new therapies.
SSc, which also can be called scleroderma, is a rare autoimmune disease. Autoimmune diseases such as SSc make your immune system over-active which causes chronic inflammation. This chronic inflammation leads to scar tissue, or fibrosis, of the skin and some internal organs.
The skin and involved internal organs with chronic inflammation from SSc become scarred over time, which makes them not work as well as they should. Lenabasum may help stop chronic inflammation, and stop scarring from getting worse without lowering the immune system.
This study is currently enrolling subjects who have scleroderma and are 18 years or older. Participation in the study will require you to complete 13 scheduled visits over the course of one year. After the completion of each visit, subjects will be compensated.
This study will assess how 18 months of oral mycophenolate will compare to 18 months of mycophenolate plus pirfenidone, in the treatment of Systemic Sclerosis related Interstitial Lung Disease. Tolerability and toxicity will also be assessed, during this study.
This research is designed to test whether combining pirfenidone and mycophenolate will result in a more rapid and possibly greater improvement in lung function than occurs when mycophenolate is used alone. While both of these drugs have been approved by the U.S. Food and Drug Administration (FDA) to treat other medical conditions, neither drug has been FDA-approved for the treatment of scleroderma related lung disease. This research is being funded by the drug company, Genentech.
Often considered as related diseases, systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) are severe autoimmune disorders characterized, among other, by dysregulation of immune cells in the blood. The roles of different immune cells in SLE and SSc remain unclear. It is of increasing importance to characterize specific immune cells and define their impact on autoimmune disease, which may lead to new therapies. The goal of this study is to identify blood immune cells associated with SLE and SSc.
This study is being conducted in order to test the safety of Brentuximab Vedotin in subjects with Diffuse Cutaneous Systemic Sclerosis. The study involves a Screening visit, a Baseline visit and 12 study visits occurring every 3 to 4 weeks, with enrollment lasting approximately 52 weeks, during which enrolled subjects will receive either the study compound or placebo.
Patients with more severe forms of scleroderma skin disease will be recruited within 7 years of the onset of their scleroderma symptoms. Two, small skin biopsy samples will be obtained from these patients. These biopsies will be placed in a culture to allow them to grow and thrive. Typically, skin biopsy tissue from patients with scleroderma produce larger amounts of skin-thickening collagen. This extra collagen leads to a process known as fibrosis, which can be noticed by patients as thickening and hardening of the skin. Potential medications will be tested in these cultures to see if they reduce the amount of skin-thickening fibrosis produced by the skin tissue from these biopsies. The overall goal of this project is to identify potential medications that could be tested later for treatment of patients with scleroderma to reduce skin fibrosis.
We have already observed that the blood cells known as monocytes from patients with the fibrotic disease scleroderma behave differently from monocytes from healthy controls. Here we will test whether patients with other fibrotic diseases also have altered monocyte function. Specifically, we will get blood from congestive heart failure and lupus patients and compare their monocytes to scleroderma patient and healthy subject monocytes. Our recent results in a mouse model for congestive heart failure suggest that we will find altered monocyte behavior in human congestive heart failure patients.
A 52 week trial to evaluate the safety and effectiveness of the study drug ifetroban versus placebo, in treating patients with diffuse cutaneous systemic sclerosis or systemic sclerosis associated Pulmonary Arterial Hypertension. Subjects over age 18, with disease duration 60 months or less, along with other inclusion criteria will receive study drug or placebo for one year, along with safety testing.
People with scleroderma often develop lung disease called interstitial lung disease. This study will use ultrasound to detect shadows on the lungs called "lung comets" among patients with scleroderma who have diagnosed lung disease, and those who have no evidence of lung disease. This is not a drug study. We are enrolling scleroderma patients to complete two visits over a period of approximately 12 months. Information about your medical history, exam findings, and laboratory tests will be collected for analysis. This study will involve approximately 40 participants.