View clinical trials related to Scleroderma, Systemic.
Filter by: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.
The primary objective of the study is to determine the activity of selexipag on Raynaud attack frequency in subjects with Raynaud's Phenomenon (RP) secondary to Systemic Sclerosis (SSc).
Doppler signals can be recorded from the lung parenchyma by means of a pulsed Doppler ultrasound system incorporating a special signal processing package; i.e. the transthoracic parametric Doppler (TPD) (EchoSense Ltd., Haifa, Israel). Systemic sclerosis patients often develop pulmonary vascular disease leading to pulmonary hypertension. The TPD system may provide important insight into pulmonary blood vessels characteristics by the LDS signals that are related to pulmonary hypertension. The TPD performance in detecting PAH in SSc patients will be assessed in the study.
Doppler signals can be recorded from the lung parenchyma by means of a pulsed Doppler ultrasound system incorporating a special signal processing package; i.e. the transthoracic parametric Doppler (TPD) (EchoSense Ltd., Haifa, Israel). Systemic sclerosis patients often develop pulmonary vascular disease leading to pulmonary hypertension. The TPD system may provide important insight into pulmonary blood vessels characteristics by the LDS signals that are related to pulmonary hypertension. The TPD performance in detecting PAH in SSc patients will be assessed in the study.
The purpose of this study is to evaluate pharmacodynamics (PD) in adult subjects with a diagnosis of Raynaud's Phenomenon secondary to Systemic Sclerosis (SSc).
The main ailm of this phase I-II study is to evaluate toxicity and efficacy of allogenic mesenchymal stem cell therapy to treat severe systemic sclerosis. In practice this treatment will be given to patients with a rapidly evolutive disease or refractory to cyclophosphamide.
Ve-cadherin is expressed in endothelial cells. Systemic slerosis is a rare auto-immune disease with a endothelial dysfunction. This study is to evaluated the level of soluble VE-cadherin and VE-cadherin antibody in patients with systemic slerosis.
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.
To investigate the ability of divalproex sodium, a histone deacetylase inhibitor, to improve the digital manifestations of scleroderma including digital edema, calcinosis cutis, digital ulcers, and joint contractures.
This is a randomized, double-blinded, clinical trial assessing the therapeutic efficacy of Botulinum toxin A (Onabotulinumtoxin A) in treating scleroderma-associated Raynaud's syndrome. Each patient will undergo injection with a treatment dose of Botulinum toxin A in one randomly-selected hand, and the contralateral hand will be injected with sterile saline (placebo) to serve as a control. Study participants at the first study visit will complete study questionnaires, their hands will be assessed clinically for digital ulceration, and their hands will undergo non-invasive laser Doppler imaging to assess blood flow. After this initial assessment, the patients will undergo peri-arterial injection of Botulinum toxin A in one hand, and of sterile saline solution (placebo) in the other, in a randomized, blinded manner. Patient will report the severity of their Raynaud's symptoms weekly over the four month study period. At one month post-injection, the patient will complete study questionnaires, their hands will be assessed clinically for digital ulceration, and their hands will undergo non-invasive laser Doppler imaging. At four months post-injection, the patient will again complete study questionnaires, their hands will be assessed clinically for digital ulceration, and their hands will undergo non-invasive laser Doppler imaging. In addition, patient will be given the option of one week post-injection visit, at which point the same assessment will be performed. At the conclusion of the study, unblinding will occur.