View clinical trials related to Scleroderma, Systemic.
Filter by:Systemic sclerosis (SSc) is a heterogeneous systemic autoimmune disease with distinct prognosis according to patients. In patients with systemic sclerosis, interstitial lung disease (ILD) concerns almost 50 % of patients and represents the main cause of mortality. Disease course in SSc-ILD is highly variable: patients can experience stable disease, slow or fast progression. Prevention of ILD progression now represents a key objective of SSc-ILD management. The understanding of the course and patterns of SSc-ILD progression is necessary, as reliable prediction tools that allow the stratification of the risk of progression. We aimed to identify the longitudinal trajectories of ILD in SSc patients using latent class mixed models and to examine their associations with SSc characteristics.
This is a randomized, double-blind, placebo-controlled, multicenter, multinational study investigating the effect of riociguat (MK-4836) in patients with early pulmonary vascular disease.
Systemic Sclerosis (Ssc) is a rare, systemic autoimmune disease characterized by skin fibrosis and vasculopathy. In addition to the skin, it is a heterogeneous disease that affects multiple organs, including the musculoskeletal, cardiac, pulmonary, and gastrointestinal systems. Patients may experience many symptoms such as pain, fatigue, dyspnea, impaired hand function, dry mouth, and difficulty sleeping. As a result of these symptoms, these patients may experience a decrease in activities of daily living, physical activity level and quality of life, while psychological problems such as anxiety and depression may increase.
This is a Phase I, single arm, open label, single center pilot study to assess a reduced-intensity conditioning regimen, bone marrow transplantation with high dose cyclophosphamide (PTCy) in recipients with refractory systemic sclerosis. This study expects to enroll 15 donor/recipient pairs for a total of 30 participants. The primary objective of this study is to assess the safety of using a reduced intensity condition (RIC) preparative regimen bone marrow transplant (BMT) with post-transplant cyclophosphamide for graft vs host disease (GVHD) prophylaxis as treatment for patients with scleroderma. Safety events are grade III-IV GVHD and treatment related mortality within 1 year. Eligibility includes patients >18 years who are eligible for transplantation according to the BMT Policy Manual, meet the 2013 ACR/EULAR Criteria for Systemic Sclerosis and display active diffuse cutaneous disease. The trial also includes analyses of the effects of BMT on skeletal and cardiac muscle using systemic scleroderma serum biomarkers of CK, aldolase, and troponin as well as periodic monitoring of circulating scleroderma auto-antibody titers, autoreactive T cells, and flow cytometric signatures over the one-year study period to correlate with response.
This study intends to carry out a prospective, multi-center cohort study based on MRI to explore the incidence of structural and functional damage of central, brain and kidney in patiant with SSc and its clinical relevance, and to search for the characteristics of serological markers of structural and functional damage of heart, brain and kidney.
The pathophysiology of systemic sclerosis (SSc) is still poorly understood and there are no effective treatments for this disease. SSc is a heterogeneous disease with varying severity. The heterogeneity of fibroblast profiles, observed in other fibrosing pathologies, has never been thoroughly explored in the skin of SSc patients. The immune system, and in particular B lymphocytes, plays a central role in the pathophysiology of SSc. The interactions between B lymphocytes and the cells responsible for excess collagen production, i.e. fibroblasts, are not fully elucidated The main objective is to analyze the heterogeneity of fibroblasts and infiltrating immune cells as well as their molecular signature in the skin of patients with SSc
The purpose of this study is to assess the safety and efficacy of tulisokibart in participants with SSc-ILD.
The aim of this project is to start a biological and clinical collection of patients presenting systemic autoimmune disease. This collection will provide appropriate biological samples to identify new biomarkers and to be accessible to the medical, scientific and industrial communities for the identification of new therapeutic strategies
People living with Systemic autoimmune rheumatic diseases (SARDs) face a new and urgent dilemma: immunosuppression increases risk for worse COVID-19 infection, yet an immune stimulation, such as vaccination, could re-activate their disease. Fear of vaccine-related disease reactivation is not of concern in other immunosuppressed groups (e.g. patients receiving chemotherapy or hemodialysis) but in SARDs, disease flare could lead to organ failure or even death. Specific research in this population is therefore critical. Moreover, among SARD patients, those on anti-CD-20 monoclonal antibody (mAb) (i.e. rituximab (anti-CD-20 mAb)), a medication used to treat inflammatory types of arthritis, have extremely low immunity post-COVID-19 mRNA vaccine. This study will test the hypothesis that a booster dose of a COVID-19 vaccine is safe and enhances post-vaccine humoral and cellular responses in SARDs patients on anti-CD-20 mAb treatment. The magnitude of this response depends on the type of COVID-19 vaccine administered and is optimal when the booster dose is a vaccine from a different group than the one used for primary immunization (mix-and-match approach).
Raynaud's phenomenon and digital ulceration are two of the most common disease manifestations leading to digital and/or toe pain in systemic sclerosis (SSc). In addition to pain, fatigue has been identified as a key stressor and the most prevalent and debilitating symptom of SSc. Both, affect significantly quality of life (QoL) domains. Pharmacological therapeutic strategies have not been proved sufficiently effective in the management of SSc-induced pain and fatigue. Evidently the effectiveness of non-pharmacological interventions (e.g., exercise, cognitive behavioural therapy) is limited, although for some of them (i.e., exercise) evidence is promising. As yet, the effects of a feasible, long-term, tailored exercise programme on pain and fatigue in people with SSc have not been explored. Therefore, the investigators propose a multicentre (n=5) research clinical trial to assess the effect of a previously established, supervised 12-week combined (aerobic and resistance training) exercise programme on pain and fatigue. The 26-month study will recruit 180 people with SSc that will be allocated randomly to two groups. Group A will perform the exercise programme parallel to standard care and Group B will receive the standard care alone. All participants will be followed for 24-weeks. Results will inform clinical practice and may improve QoL for people with SSc.