View clinical trials related to Scleroderma, Systemic.
Filter by:Many patients with Scleroderma (Systemic sclerosis) experience damage to blood vessels, mainly to the small arteries. A common manifestation of this is Raynaud's phenomenon (fingers or toes turning white then blue in the cold) and digital ulcers (open sores on the fingertips). The purpose of this study is to see how effective the study drug Human Factor XIII Concentrate is in treating patients who have these and other common manifestation of Scleroderma. It will be given in addition to the accepted treatments used for this disease.
Esophagus is commonly affected in Systemic Sclerosis (SSc) and esophageal function is compromised in about 75% of patients. Previous studies have shown that buspirone, an orally available 5-HT1A (serotonin 1a) receptor agonist, enhances esophageal motility in healthy volunteers. Recently, the investigators observed that a single dose of buspirone (10mg) improves lower esophageal sphincter (LES) function in patients with SSc and esophageal involvement . Objectives: To evaluate the effect of continuous administration of buspirone on esophageal dysfunction and related symptoms in SSc using high resolution manometry (HRM).
Currently investigators do not have diagnostic and prognostic markers for SSc which almost always starts with a vascular disease (Raynaud's disease) isolated for several years. The primary purpose is to highlight discriminating metabolic profiles depending on the characteristics of the disease, allowing early diagnosis of SSc at the onset of vascular lesions, by comparing the profiles of SSc beginners (<3 years) to established forms (> 3 years). Secondary purposes: - Prognosis: to study the metabolomics profile of SSc when a visceral complication occurs - Diagnosis: to compare the metabolomics profile of SSc to undifferentiated connective tissue disease (UCDT), Raynaud's disease (RD), vascular disease (VD) and healthy controls - Exploratory: to compare the metabolomics profile of blood, urine and skin of SSc patients
To assess the effectiveness of Autologous Hematopoietic Stem Cell transplantation (AHSCT) for early severe or rapidly progressive Systemic Sclerosis (SSc) as currently performed by different study protocols used across Europe in various EBMT centres through the careful recording and analysis of routinely collected clinical and biological data.
This prospective single-center study evaluates a current care procedure. It includes 14 patients diagnosed with SSc according to the ACR/EULAR criteria or the Leroy & Medsger criteria of the disease. Patients are enrolled if they wish for a therapeutic care of their face, have a Mouth Handicap in Systemic Sclerosis (MHISS) score greater than 20 (scale 0-48), a modified Rodnan skin score upper or equal to 1 on the face (scale 0-3), and a mouth opening less than 55 millimeters. They should not have anticoagulant, anti-platelets aggregation medication or a daily steroid dose upper 20mg per day. Their BMI should exceed 17. Micro fat grafting is a minimally invasive and usual procedure performed under local anesthesia. Fat tissue is harvested (around 50 milliliters) using a 14 gauge or 2 mm diameter cannula from areas around the knees, the abdomen or the hips under a gentle aspiration. Lipoaspirated fat is then filtrated by the PureGraft system that offers a sterile, closed, single-use system, leading to a fast, consistent and controlled preparation. Then, 10 to 25 milliliters of this purified fat product is transferred through a 21 gauge or 0.8 mm diameter cannula in two or four points of the face, with the entry points located around the mouth.
Scarring of the lungs is common in patients with scleroderma and is one of the main causes of death. Patients with scleroderma very frequently have problems with their gullet (esophagus), the food pipe that leads into the stomach. Normally, a small circular muscle at the base of the esophagus opens to allow food to pass into the stomach and closes to keep the digestive fluids from flowing back up into the gullet. In patients with scleroderma, the muscle may become weak and no longer close properly. Gastroesophageal reflux (GER) is the medical term for reflux of stomach contents into the esophagus. Our hypothesis is that small amounts of GER can move back up into the esophagus and get inhaled into the lungs, and may be one of the triggers for lung scarring. We propose to look for certain substances normally only found in the stomach in the "exhaled breath condensate" which is collected by breathing comfortably into a cooled cylinder, allowing the breath to condensate. In a smaller group of patients, we also plan to perform a bronchoalveolar lavage, a more widely studied test in which a small amount of fluid is introduced into a small part of the lungs through a fine tube, and then removed for examination, to evaluate whether the two tests provide similar measurements. We will also evaluate the correlation between these molecules and other tests, including lung function, and markers of lung scarring activity, and tests to look at how the esophagus is working so that we can get a clearer picture of how this affects patients' daily lives. Finally, we will be following up patients over time with lung function to see whether evidence of GER into the lungs is linked with a greater likelihood of worsening of lung scarring in the future.
The investigators purposes are to define the prevalence of omeprazole resistance gastroesophageal reflux disease (GERD) in systemic sclerosis (SSc), to compare the efficacy of omeprazole in combination with algycon versus omeprazole in combination with domperidone on the severity of reflux symptoms in omeprazole resistant GERD in SSc, and to compare the efficacy of omeprazole in combination with algycon versus omeprazole in combination with domperidone on the frequency of symptoms in omeprazole- resistant GERD in SSc.
Systemic sclerosis (SSc) is an orphan, multiorgan disease affecting the connective tissue of the skin and all internal organs. Interstitial lung disease is a frequent morbidity and mortality-driving manifestation in systemic sclerosis. This observational trial (OT) is part of the collaborative project "DeSScipher", one out of five OTs to decipher the optimal management of systemic sclerosis. Aim of this observational try is to identify: - The state of clinical practice in Europe for prevention and treatment of interstitial lung disease and its impact on lung function and disease progression - The potential predictors and confounders for response to therapy
Systemic sclerosis (SSc) is an orphan, multiorgan disease affecting the connective tissue of the skin and several internal organs. Pulmonary hypertension (PH) is a fatal disorder characterized by an increase in pulmonary vascular resistance, which leads to right ventricular failure. Despite being recently the object of greater attention and despite therapeutic advances, pulmonary hypertension due to SSc remains associated with a dismal 47 - 67% 3-year survival. Among SSc patients prospectively followed in the "European League Against Rheumatism Scleroderma Trials and Research" (EUSTAR) cohort, 26% of death was related to pulmonary hypertension. Although some previous data have suggested the protective effects of calcium channel blockers on the development of pulmonary hypertension, the potential preventive effects of vasodilators for the prevention of Pulmonary hypertension have not been determined yet. In addition to be considered routinely for the treatment of SSc-related pulmonary hypertension, prostanoids, endothelin receptor antagonists (ETRA) and Phosphodiesterase-5 inhibitors (PDE5i) can also be used for this indication. This observational trial is one out of five observational trials of the collaborative project "To decipher the optimal management of systemic sclerosis" (DeSScipher). Aim of this observational trial is: - to compare the outcomes of adult and juvenile SSc patients who are at high risk of developing pulmonary hypertension and are receiving either different vasodilator treatments or no vasodilator treatment.
Systemic sclerosis is an orphan, multiorgan disease affecting the connective tissue of the skin and several internal organs. Digital ulcers are frequent and have a major impact on the quality of life in patients with systemic sclerosis. The etiology of digital ulcers is complex and multifactorial and the principal mechanisms underlying the digital ulcers formation are ischemic, mechanic and inflammatory, alone or in combination, on the basis of the systemic sclerosis vasculopathy. Consequently, there are at least three types of DU: (i) those localized at the tips of the fingers and toes, mainly resulting from an ischemic process, (ii) those localized on the dorsal aspect of the fingers where the skin retraction due to fibrosis over bony prominences seems to be the main cause, and (iii) those evolving on a pitting scar or subcutaneous calcinosis due to a combined irritative-inflammatory mechanism. An early therapy to prevent or rapidly heal digital ulcers is today considered a mandatory approach to maintain quality of life and spare the enormous costs due to conventional digital ulcer management. This observational trial is part of the collaborative project "DeSScipher", one out of five observational trials to decipher the optimal management of systemic sclerosis. Aim of this observational trial is: 1. To identify the best treatment combination for prevention of digital ulcers in patients with fulfilment of the new ACR/EULAR SSc criteria or the preliminary VEDOSS criteria for very early diagnosis of systemic sclerosis 2. To identify the best treatment associated with improved healing of digital ulcers in patients with fulfilment of the new ACR/EULAR SSc criteria Thus, the observational trial consist of a prevention arm and a healing arm.