View clinical trials related to Scleroderma, Localized.
Filter by:To determine sleep disturbance, pulmonary function test, 6 minute walk, echocardiogram abnormality, arterial blood gas, ventilation/perfusion scan of the chest in Scleroderma patient with Pulmonary Hypertension.
Systemic Sclerosis (Scleroderma) varies greatly in clinical manifestations, mode of presentation, and course. The natural history of this chronic autoimmune disease ranges from benign to fatal. Patients are classified into limited and diffuse scleroderma defined by the degree of skin involvement. Patients with limited disease (e.g. the C.R.E.S.T. syndrome) generally have mild disease and normal survival. However, patients with diffuse cutaneous scleroderma often have severe multi-system disease that is not only devastating emotionally and physically but is associated with a 60-70% five year survival and a 40-50% 10 year survival. No therapies have proven effective in the treatment of scleroderma. Strategy to treat scleroderma have included attempts to prevent fibrosis with drugs that interfere with collagen metabolism, attempts to modify the disease process by immunosuppression and attempts to alter the disease by vasoactive drugs. High dose of corticosteroids and other immunosuppressive drugs (e.g. chlorambucil, 5-fluorouracil, methotrexate, cyclophosphamide, cyclosporine) used at conventional doses have not proven curative, but have shown some benefit for inflammatory features of the disease (e.g. arthritis, myositis, fibrosing alveolitis). Both allogeneic and autologous bone marrow transplantation (BMT) have shown to modify and in some instances reverse a variety of animal models of autoimmune disease. This has prompted many investigators to propose the use of peripheral blood stem cell transplantation (PBSCT) for the treatment of autoimmune disease including scleroderma. Unfortunately, this approach risks infusing untreated autoreactive lymphocyte clones after the immunoablative preparative regimen. We have previously demonstrated that high-dose cyclophosphamide without BMT can induce durable and complete remissions in another autoimmune disease, severe aplastic anemia. Recent data with high dose cyclophosphamide show that it can induce complete remissions in other autoimmune hematologic disorders. The objective of this study is to determine whether high dose cyclophosphamide can induce a durable remission in scleroderma patients with life-threatening disease, and to determine toxicity of high dose cyclophosphamide in high risk scleroderma patients.
In vitro studies have shown that imatinib 1mM inhibits strongly the growth of cutaneous fibroblasts. The hypothesis is that imatinib inhibits PDGFR which is known to be a potential target for the molecule, as recently also proposed after the discovery of autoantibodies activating the PDGF receptors. Recent data indicate that TGFb is also a potential target of imatinib. Cutaneous scleroderma is characterized by progressive cutaneous fibrosis caused by hyperactive dermal fibroblasts. Since no established treatment for skin sclerosis in scleroderma is currently available. This study will test the safety and efficacy of imatinib in the treatment of patients with scleroderma and severe cutaneous involvement.
Systemic sclerosis (scleroderma) is an autoimmune connective tissue disease that involves the skin and other internal organs for which there are few effective treatment options. We hypothesize that treatment with abatacept, a new therapy recently approved for the treatment of rheumatoid arthritis, may reduce the progression of skin thickening and fibrosis in people with scleroderma.
The purpose of this study is to determine the timeline of progression from pre-pulmonary hypertension to diagnosable pulmonary hypertension based on right heart catheterization. Moreover, to determine the timeline for progression from diagnosable pulmonary hypertension to clinical worsening of disease as defined as death, hospitalization, or worsening of PHT symptoms.
The purpose of this study is to determine whether the drug Bosentan improves exercise tolerance in scleroderma patients.
The purpose of this trial is to study the proportion of scleroderma patients who suffer from asymptomatic coronary atherosclerosis compared to healthy controls.
Researchers from the Division of Pulmonary and Critical Care Medicine at University of California, San Francisco (UCSF) are conducting a study to evaluate whether mycophenolate mofetil (an immunosuppressive medication, trade named CellCept) is safe and effective for preventing the lung damage from scleroderma from getting worse.
Localized scleroderma or morphea is a fibrotic disease, imiquimod induces cytokines which inhibit fibrotic activity . We propose that topical imiquimod 5% cream is efficacious and safe in treating plaque-type morphea. Twenty adults will be enrolled for a pilot study. A screening and baseline clinic visit will be required for each patient enrolled in the study. Each visit will involve completing a medical history, skin examination, digital pictures, histologic examination if the patient consents and an ultrasonographic score. One morphea plaque will be treated with topical imiquimod 5% cream, and another morphea lesion with vehicle cream. Patients will be asked about side effects (local and systemic). Patients will be followed up in the clinic at 3, 6 and 9 months of therapy, and 3 months following end of treatment period (month 12).
Scleroderma is an autoimmune disease of unknown origin. Recently, the role of environmental factors, and particularly toxic drug exposure, in the genesis of scleroderma has been suggested. This prompted us to conduct this prospective, case-control, multicentric study, including 2 groups of subjects: - 100 patients with scleroderma - 300 sex- and age-matched healthy controls. The aim of our study is to determine whether exposure to toxics is higher in patients with scleroderma compared with healthy controls.