Scleroderma is a complex disease characterized by activation of the immune system, small-vessel vasculopathy, and fibrosis of the skin and other organs. This review is limited to the discussion of scleroderma fibroblast biology and the cellular and molecular mechanisms that contribute to the abnormal deposition of collagen. Selected aspects of abnormal extracellular matrix (ECM) regulation by s...

Functional and structural vascular lesions have been observed in the organs involved in scleroderma. The etiology of these vascular changes is poorly understood. The ability to isolate, characterize, and maintain endothelial cells in vitro provides a target cell population to study endothelial damage in scleroderma. The present report describes the effect of scleroderma serum on endothelial, sm...

introduction: pulmonary involvement secondary to systemic sclerosis (ssc) is the major cause of morbidity and mortality in ssc patients. we designed this study to determine the correlation of important lung function parameters with lung high resolution ct (hrct) scan findings.materials and methods: thirty–two consecutive diffuse ssc patients with pulmonary fibrosis were enrolled in this cross-s...

Approximately 20% of patients with the limited form of scleroderma will develop pulmonary hypertension which is generally a late stage fatal complication. Why pulmonary hypertension occurs in this subset of patients is unknown and it has not been possible to predict which patients are at risk. Nailfold capillary dilatation, distortion and drop occurs universally in patients with scleroderma and...

OBJECTIVES To evaluate the presence of ET-1 in patients with scleroderma and its correlation with the level of disease activity; to verify if the levels of endothelin are associated with the clinical profile and autoantibodies of scleroderma, and even if there is an association with microvascular injury detected by nailfold capillaroscopy. METHODS A total of 74 patients, 37 patients with scle...

The prototypic autoimmune diseases involving skin (lupus, dermatomyositis) typically result in epithelial injury and autoantibodies to characteristic cellular antigens. Disease-specific autoantibodies are also found in scleroderma, but scleroderma is different from other cutaneous autoimmune diseases because epithelial injury does not occur. Multiple factors and combinations of factors (immune ...

Scleroderma is a disease characterized by immune activation, vasculopathy, fibroblast stimulation, and connective tissue fibrosis. End-organ damage occurs due to progressive tissue fibrosis and vasculopathy. Markers of incipient vasculopathy have not been well studied in scleroderma. However, reduced renal functional reserve and proteinuria are common indicators of progressive vasculopathy in d...

BACKGROUND The scleroderma is a complex autoimmune collagen disorder that can affect many organs simultaneously, as it occurs in the systemic sclerosis (SS), or only the skin, as it occurs in the localized scleroderma (LS). The neurological presentation is extremely uncommon, and even more uncommon are the symptoms of the scleroderma in the cerebellum. CASE DESCRIPTION We report the case of a...

Scleroderma is a chronic systemic autoimmune disease (primarily of the skin) characterized by fibrosis (or hardening), vascular alterations and autoantibodies production.There are currently no effective therapies against this devastating and often lethal disorder. Despite the interest for the immunomodulatory effects of mesenchymal stem cells (MSCs) in autoimmune diseases, the role of MSCs in s...