View clinical trials related to Scleroderma, Systemic.
Filter by: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. In addition to medical treatment, rehabilitation programs for the patient are an important part of treatment to eliminate or reduce these symptoms and their consequences. Many problems such as time and resource constraints, transportation problems prevent access and compliance with the rehabilitation program. Also; For the coronavirus disease 2019 (COVID-19) epidemic that emerged in Wuhan, China in 2019, many countries have implemented many practices such as social distance, mandatory quarantine and transportation restrictions in order to better control the spread of the virus. Many people with SSc are at risk of serious complications from COVID-19 if infected due to lung involvement (>40% have interstitial lung disease) and widespread use of immunosuppressant drugs. Most countries have recommended that people with medical conditions such as SSc undergo strict isolation during the COVID-19 pandemic. As a result, patients' access to the rehabilitation program became more difficult in this process. In addition, social isolation due to the COVID-19 outbreak may increase physical inactivity and cause complications that may develop accordingly. When the literature was examined, no studies were found showing the effect of telerehabilitation program on anxiety depression, physical activity, sleep, fatigue and quality of life in patients with SSc.
Scleroderma (or Systemic Sclerosis - SSc) is one of the most neglected diseases worldwide, according to the World Health Organization. In the adult population with SS, the systemic effects of the disease, such as respiratory and peripheral muscle dysfunction, cause a decrease in quality of life. As a consequence, there is a concern about functional rehabilitation, since the aging of this population is already a reality. Thus, the objective of this project is to evaluate the effects of functional rehabilitation on functional capacity and quality of life in women over 18 years of SS. In this longitudinal intervention study, patients will be submitted to a three-month rehabilitation program. Before and after the intervention, patients will be submitted to the following assessments: Cochin Hand Functional Scale (CHFS), Short-Form 36 Health Survey (SF-36); Scleroderma Health Assessment Questionnaire Disability Index (SHAQ-DI); lung function; lung ultrasound; handgrip; Glittre Activities of Daily Living test. Thus, it is expected that patients with SS will benefit significantly, with a consequent improvement in musculoskeletal function and , functional capacity and health-related quality of life.
This single-center, prospective, open-label, quasi-experimental, intra-individual comparative study will include a consecutive cohort of 21 patients with diffuse or limited, minimally active scleroderma with 3 to 30 years of evolution. Patients will have 4 sessions of pulsed dye laser 595 nm spaced 8 weeks apart. The final quadruple evaluation by several evaluators will be 2 months after the last session, on the following criteria: evolution of the number of telangiectasia; subjective improvement score (LINKERT scale); impact on quality of life (SKINDEX score); visual analog pain scale (VAS); adverse events (AEs), including discontinuation of treatment due to post-session purpura (AT-PPS); patient satisfaction (yes or no).
A subset of autoimmune diseases (ADs) in children and young adults are life-threatening and unresponsive to conventional treatments. In these patients, the delivery of high dose immunosuppressive therapy followed by autologous stem cell transplant (ASCT) offers a treatment strategy capable of purging the pathogenic, autoreactive immune system and an opportunity for "immune reset." This strategy has been used in adults across a myriad of indications with evidence for efficacy. This study proposes a pilot study to evaluate this therapeutic strategy in children and young adults with systemic sclerosis (SSc) and systemic lupus erythematosis (SLE), two potentially life threatening autoimmune diseases that may response to this therapeutic approach.
This trial will study the safety and efficacy of intravenous infusion of cultured allogeneic adult umbilical cord derived mesenchymal stem cells for the treatment of Systemic Sclerosis
A complex interaction between demographic, environmental and genetic mechanisms impact the onset, severity and outcome of ILD-SARDs through dysregulation of the immune system and lung pro-biotic pathways. Comorbidity and genetic risk indicate that there are overlapping pathogenic mechanisms among SARDs, some of which underlie ILD in different SARDs. The purpose of this biobank is to study the clinical, pathological, laboratory, and imaging characteristics of SARDs patients with lung involvement. This will help identify as unique features underlying lung involvement in SARDs. In addition, this may lead to the discovery of novel mechanisms of disease and potentially novel targets of treatment for SARDs patients with lung disease.
This is a randomized, multi-site, adaptive, open-label clinical trial comparing the immune response to different additional doses of COVID-19 vaccine in participants with autoimmune disease requiring IS medications. All study participants will have negative serologic or suboptimal responses (defined as a Roche Elecsys® Anti-SARS-CoV-2 S result ≤200 U/mL) or a low immune response (defined as a Roche Elecsys® Anti-SARS-CoV-2 S result >200 U/ml and ≤2500 U/mL) to their previous doses of COVID-19 vaccine. The study will focus on 5 autoimmune diseases in adults: - Systemic Lupus Erythematosus (SLE) - Rheumatoid Arthritis (RA) - Multiple Sclerosis (MS) - Systemic Sclerosis (SSc), and - Pemphigus. This study will focus on 4 autoimmune diseases in pediatric participants: - Systemic Lupus Erythematosus (SLE) - Juvenile Idiopathic Arthritis (JIA) - Pediatric-Onset Multiple Sclerosis (POMS) - Juvenile Dermatomyositis (JDM)
Systemic sclerosis (SSc) is a rare connective tissue disease characterized by a high clinical heterogeneity with unpredictable evolution that could engage functional and life-threatening prognosis. Most of patients develop skin fibrosis gradually spreading. Two clinical distinct forms are described according to the extension of skin fibrosis: limited (lSSc) and diffuse (dSSc) SSc. It is now admitted that a spreading of skin fibrosis is associated with poor prognosis. This disease can be complicated by pulmonary arterial hypertension (PAH), interstitial lung disease (ILD) and scleroderma renal crisis (SRC) representing the main causes of death in SSc. Usually, PAH is associated with lSSc, and ILD and SRC are associated with dSSc. Prognosis is also different regarding skin phenotype with a higher mortality rate in dSSc. An easy score, called modified Rodnan skin score, is commonly used in clinical practice to evaluate the spreading and severity of skin fibrosis, but this score is hardly reproducible. Ultrasound can be used to measure skin thickness and is more reproducible than the Rodnan skin score. Nevertheless, non-invasive analysis of fibrosis composition in different areas is not possible with these two technics. Thus, in this study we will investigate Raman spectroscopy, a non-invasive technic based on the interaction of a low-intensity laser with matter.
Innate T cells (ITC) are decreased in systemic sclerosis (SS) and an early lymphocyte innateness has been reported. In the other part, ITC are implicated on inflammatory process, including the IL-33/ST2 axis, which is also involved in ScS endotheliopathy. Data are however scarce and physiopathological mechanisms have not been assessed to date. The investigators hypothesize a global lymphocyte innateness in SSc, linked to a chronic ITC stimulation by innate signals leading to ITC exhaustion, and their potential role in endotheliopathy and fibroblast activation in SSc.
The purpose of this study is to assess feasibility, safety and preliminary efficacy of Extracorporeal Photopheresis in the treatment of active diffuse cutaneous systemic sclerosis (dcSSc). This pilot study will help to determine if further study (a RCT) is justified.