Active clinical trials for "Scleroderma, Systemic"

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: 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 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.

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.

Migration Inhibitory Factor has proliferative and antiapoptotic actions on fibroblasts which may be relevant to scleroderma because of the central role of a dysregulated fibroproliferative response in disease-affected tissues

The aim of this study was to assess serum N-terminal proBNP (NT-proBNP) in systemic sclerosis patients and to establish whether it reflects the severity of RV overload.

To explore the association among TCM pattern, TCM tongue diagnosis and TCM pulse diagnosis for Autoimmune disease and Dry eye syndrome

The purpose of this study is to assess the value in terms of sensitivity, specificity and likelihood ratio of the stress echocardiography in the screening of pulmonary arterial hypertension in patients with systemic sclerosis and indirect signs of pulmonary arterial hypertension.

This phase I/II pilot trial seeks to demonstrate that prolonged administration of Campath-1H without prior marrow or stem cell harvesting can result in immunoablation similar to that achieved by hematopoietic stem cell transplantation (HSCT) from either bone marrow or peripheral blood stem cell sources in children and adolescents with severe treatment refractory systemic sclerosis (SSc).

The primary objective of the study is to assess the change in systemic sclerosis (SSc)-associated gastrointestinal (GI) tract symptoms over a 1-year period in participants with SSc.

Cuprimine (penicillamine) was made available in the Philippines by the Sponsor under a Compassionate Special Permit issued by the Bureau of Food and Drugs. Physicians were able to request the drug for their patients from the Sponsor. A Clinical Study Report form was completed for each purchase of Cuprimine.

To establish a new prospective cohort of Korean patients with systemic sclerosis and track the natural history of the disease over time. To generate new hypotheses for further investigation.