View clinical trials related to Systemic Scleroderma.
Filter by:This is a single arm study to evaluate the efficacy and safety of CD19 targeted CAR-T cells therapy for patients with Refractory Autoimmune Disease
The study is a randomized, double-blind, placebo-controlled clinical study of the efficacy and safety of divozilimab in patients with systemic scleroderma.The study will enroll adult patients of both sexes diagnosed with active systemic scleroderma according to the ACR/EULAR 2013 criteria with a modified Rodnan skin score (mRSS) of 10 to 20. In patients having signs of ILD, the Forced Vital Capacity (FVC) should be at least 40 % of the due value. Subjects will be randomized to divozilimab or placebo group.
Scleroderma (SSc) is an autoimmune disease characterized by fibrosis (or collagen deposition) of the skin and internal organs. The extent of skin fibrosis is an important predictor of internal organ complications and increased mortality. Currently imprecise and subjective methods that varies amongst different doctors for the same patient are available to quantify skin fibrosis in patients, by "pinching" their skin and assessing how thick it is; this is the method used to determine the modified Rodnan skin score (mRSS). Skin thickness and the amount of fibrosis can change over time due to disease progression or in response to therapy. In this research, longitudinal measurements will be taken to determine if spatial frequency domain imaging (SFDI) can detect changes in skin thickness that occur over time in response to therapy or from disease progression in scleroderma patients. This study will compare SFDI with other clinical outcome assessments of skin thickness and fibrosis in scleroderma patients including mRSS, skin biopsy histology, scleroderma skin patient reported outcome (SSPRO), ultrasound, and durometry (durometer measures skin hardness). SFDI information will also be compared with capillaroscopy (allows for non-invasive imaging of the nailfold capillaries) if available from the electronic medical record. If SFDI correlates well with other clinical outcome assessments, it may be used in the future as a rapid, non-invasive tool for monitoring disease activity in scleroderma patients.
This early phase I trial studies the side effects and feasibility of total body irradiation using intensity modulation radiation therapy (IMRT) when given in combination with cyclophosphamide prior to stem cell transplant to treat severe systemic sclerosis. IMRT delivers total body radiation therapy more precisely and may reduce radiation exposure to sensitive normal organs. Giving chemotherapy, such as cyclophosphamide, and total body irradiation before a donor stem cell transplant helps kill cancer cells in the body and helps make room in the bone marrow for new blood-forming cells (stem cells) to grow. Giving IMRT and cyclophosphamide prior to stem cell transplant may work better in treating severe systemic sclerosis and reduce radiation doses to lung and kidneys compared to cyclophosphamide alone.
This phase Ib trial studies the side effects of nivolumab and to see how well it works in treating patients with autoimmune disorders and cancer that has spread to other places in the body or cannot removed by surgery. Immunotherapy with monoclonal antibodies, such as nivolumab, may help the body's immune system attack the cancer, and may interfere with the ability of tumor cells to grow and spread.
Systemic Scleroderma (SCS) is an autoimmune disease characterized by vascular involvement, a dysimmune condition, cutaneous and visceral fibrosis. Interstitial lung disease (ILD) affects 75% of SSc patients and is the leading cause of death in SSc. No diagnostic or prognostic biomarkers of SSc-associated ILD have been validated to date. The search for such a serum biomarker is essential to assess the severity of these patients and to help the therapeutic management. We have shown that soluble fractalkine is elevated in SSc patients, especially in SSc patients with ILD. The fractalkine is both an endothelial adhesion molecule and a chemokine that binds to the CX3CR1 receptor expressed by immune populations. It would thus reflect the vasculopathy and inflammation that lead to the fibrosing pulmonary involvement of this disease. Objectives and means: We aim to perform a low-risk interventional biomedical research which main objective is the quantitative evaluation of soluble fractalkine in SSc patients with ILD in comparison with SSc patients without ILD. This epidemiological, explanatory, analytical, single-center study will comprise three groups: 1 / SSc without ILD (control group in the context of SSc), 2/ SSc with ILD and 3/ patients with idiopathic pulmonary fibrosis (IPF) (control group of the ILD). Secondary objectives are evaluation of: 1 / fractalkine levels in the IPF, 2 / correlations between fractalkine levels and severity of ILD and of SSc disease over time, 3 / correlations between fractalkine and 2 other biomarkers: KL-6 (marker of pulmonary fibrosis) and soluble CD146 (sCD146, marker of vasculopathy), 4 / predictive values of the decline in lung function of these 3 markers.