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Full-Text Articles in Dermatology
Endothelial To Mesenchymal Transition (Endomt) In The Pathogenesis Of Human Fibrotic Diseases., Sonsoles Piera-Velazquez, Fabian A. Mendoza, Sergio A. Jimenez
Endothelial To Mesenchymal Transition (Endomt) In The Pathogenesis Of Human Fibrotic Diseases., Sonsoles Piera-Velazquez, Fabian A. Mendoza, Sergio A. Jimenez
Jefferson Institute of Molecular Medicine Papers and Presentations
Fibrotic diseases encompass a wide spectrum of clinical entities including systemic fibrotic diseases such as systemic sclerosis, sclerodermatous graft versus host disease, nephrogenic systemic fibrosis, and IgG₄-associated sclerosing disease, as well as numerous organ-specific disorders including radiation-induced fibrosis, and cardiac, pulmonary, liver, and kidney fibrosis. Although their causative mechanisms are quite diverse, these diseases share the common feature of an uncontrolled and progressive accumulation of fibrous tissue macromolecules in affected organs leading to their dysfunction and ultimate failure. The pathogenesis of fibrotic diseases is complex and despite extensive investigation has remained elusive. Numerous studies have identified myofibroblasts as the cells …
Endothelial To Mesenchymal Transition (Endomt) In The Pathogenesis Of Systemic Sclerosis-Associated Pulmonary Fibrosis And Pulmonary Arterial Hypertension. Myth Or Reality?, Sergio A. Jimenez, Sonsoles Piera-Velazquez
Endothelial To Mesenchymal Transition (Endomt) In The Pathogenesis Of Systemic Sclerosis-Associated Pulmonary Fibrosis And Pulmonary Arterial Hypertension. Myth Or Reality?, Sergio A. Jimenez, Sonsoles Piera-Velazquez
Department of Dermatology and Cutaneous Biology Faculty Papers
Systemic Sclerosis (SSc) is a systemic autoimmune disease characterized by progressive fibrosis of skin and multiple internal organs and severe functional and structural microvascular alterations. SSc is considered to be the prototypic systemic fibrotic disorder. Despite currently available therapeutic approaches SSc has a high mortality rate owing to the development of SSc-associated interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH), complications that have emerged as the most frequent causes of disability and mortality in SSc. The pathogenesis of the fibrotic process in SSc is complex and despite extensive investigation the exact mechanisms have remained elusive. Myofibroblasts are the cells …