View clinical trials related to Scleroderma, Diffuse.
Filter by: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.
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.
Systemic sclerosis (SSc) is the most severe of the systemic autoimmune diseases. It is characterized by skin and organ fibrosis (mainly interstitial lung disease, which affects 40-50% of patients), as well as severe vascular complications such as pulmonary hypertension (5-10%), renal crisis (2%), and digital gangrene (5%). There are currently no validated prognostic biomarkers for the progression of SSc, yet it is crucial to better predict the progression of SSc to optimize patient management, but also to identify the optimal population for clinical trials ("progressor" patients). Furthermore, there are no validated biomarkers of response to immunosuppressive therapies that would be useful both in patient management and in the evaluation of new treatments in clinical trials. The internal medicine department of the Lille University Hospital is a national and European reference center for the management of patients with SSc. Nearly 500 patients are followed annually in the internal medicine department. As part of their routine care, patients are hospitalized in average once a year in the internal medicine department of the Lille University Hospital for a complete assessment of their SSc. This assessment includes a detailed medical observation, complementary examinations and blood and urine biology tests. The purpose of this study would be to collect 2 additional blood samples during the standard evaluation of scleroderma patients. The main objective of this collection of biological samples for scientific research will be the identification of new biomarkers associated with prognosis and treatment response to improve the management of SSc patients.
The purpose of this study is to assess the effect of transcutaneous auricular vagus nerve stimulation (ta-VNS) on gastrointestinal symptoms and quality of life in systemic sclerosis (SSc) patients.