View clinical trials related to Scleroderma, Systemic.
Filter by:This is a randomized, blinded, and controlled trial to assess the efficacy and safety of UVA1 phototherapy in the treatment of active morphea in adults and children. Forty patients will be randomized to receive either medium dose (70 J/cm2) phototherapy (active UVA1 phototherapy) with an ultraviolet translucent acrylic screen or "sham" UVA1 (0 J/cm2) phototherapy with an ultraviolet opaque acrylic screen 3 times per week for 10 weeks. The phototherapists, patients, and principal investigator will be blinded to whether the patients receive active or sham UVA1 phototherapy. Patients will only be allowed to apply emollients during the study. Patients completing the randomized placebo controlled trial (RPCT) will be followed during an open observation period for 3 months. During the open phase, all outcome measures from the RPCT (LoSSI, PGA-A) will be assessed every 5 weeks as well as adverse events. Patients who received sham UVA1 phototherapy will be invited to receive active UVA1 phototherapy using the same protocol as in the RPCT during the open observation. Adult patients enrolled in the RPCT will also be part of a nested translational study investigating the effect of UVA1 phototherapy on gene expression from whole skin biopsies taken before (Study Visit 1) and after UVA1 phototherapy (Study Visit 3). Gene expression profiles will be compared in lesional skin before and after treatment as well as nonlesional skin.
The primary intent of this study is to add to the body of knowledge on scleroderma patients with interstitial lung disease. While lung disease is recognized as the leading cause of death amongst patients with scleroderma, there is not a large body of literature describing the long-term morbidity and mortality rate of these scleroderma patients. For this reason, the investigators are following participants of the Scleroderma Lung Study (NCT00004563) after their participation in that study was concluded. In addition, the investigators will assess if the subjects who received one year of oral cyclophosphamide in the Scleroderma Lung Study experienced progression of their scleroderma-related lung disease following the end of the study.
The purpose of this study is to determine whether rituximab is effective in the treatment of articular symptoms that occur in systemic sclerosis related polyarthritis
The aim of the study is to determine if postocclusive hyperemia of palmar and dorsal face of the hand with Laser speckle contrast imaging discriminate between patients with systemic sclerosis, subjects with primary Raynaud's phenomenon and healthy subjects.
Pulmonary arterial hypertension (PAH) is a condition characterized by an increased pulmonary vascular resistance that can lead to right heart failure and death. Several diseases are known etiologies of PAH including scleroderma and cirrhosis. The presence of PAH in the context of systemic sclerosis or cirrhosis has a dramatic impact on prognosis and survival of the connective tissue or liver disease. Despite advances in the diagnosis of PAH, echocardiography remains a necessary test for screening PAH in patients with scleroderma or cirrhosis. However, echocardiography is less than ideal for diagnosing PAH and predicting treatment response. Thus, there is a pressing need to identify methodologies that can accurately and non-invasively recognize the presence of PAH in patients with scleroderma and cirrhosis. Hypothesis: 1. To measure endothelial function and exhaled gases in patients with scleroderma and cirrhosis. To assess whether they correlate with the presence or the development of PAH. 2. The degree of local (forearm) capillary vasodilation during treprostinil iontophoresis identifies patients who will develop PAH and in those already diagnosed PAH predicts response to PAH-specific therapies.
This CARRA Registry study will create a foundational database for rheumatic diseases of childhood using a novel informatics infrastructure developed as part of the larger clinical project. The creation of a CARRA-wide informatics infrastructure will enable efficient, observational, disease-related data capture across all CARRA sites for pediatric rheumatic diseases. The CARRA Registry study will demonstrate the feasibility of expanding to more data intensive registries for observational studies, comparative effectiveness research, pharmaceutical clinical trials and translational research.
One of the major problems of systemic sclerosis (SSc) patients is suggested to be articular involvement. Mostly involved joints in SSc were reported as wrist, carpometacarpal-interphalangeal, foot, knee, hip and shoulder however there have been little knowledge on sacroiliac joint. Here the investigators plan a study on the involvement of this joint in SSc.
This is a 48 week, phase IIa, single center, randomized, double-blind, placebo-controlled, proof-of-concept pilot study. All participants will first be treated with mycophenolate mofetil (MMF, Cellcept) and titrated up to a dose of 2 grams/day. Following this period, half will be given either a belimumab (Benlysta®) or placebo intravenous infusion to treat early diffuse cutaneous systemic sclerosis. Belimumab/MMF is expected to improve disease activity measured by an improvement in skin thickening and stability of pulmonary function test measurements when compared to patients treated with placebo/MMF.
This is a mechanistic research study to evaluate the relationship between cough, reflux, and aspiration in patients with systemic sclerosis (scleroderma).
Primary Objective: - To evaluate safety and tolerability of 8-week oral administration of SAR100842 in patients with diffuse, cutaneous systemic sclerosis. Secondary Objectives: - To evaluate the pharmacodynamic effect of SAR100842 in patients with systemic sclerosis as measured by disease related biomarkers and Lysophosphatidic acid (LPA) receptor signaling markers in blood and skin - To explore the effect of SAR100842 on skin thickness in patients with systemic sclerosis as measured by the Modified Rodnan Skin score (mRSS) - To explore the effect of SAR100842 on quality of life as measured by the Scleroderma Modified Health Assessment Questionnaire (SHAQ).