The pathogenesis of fibrosis and renal disease in scleroderma: Recent insights from glomerulosclerosis |
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Authors: | Sungchun Lee Sohee Lee Kumar Sharma |
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Institution: | (1) Dorrance Hamilton Research Laboratories, Division of Nephrology, Department of Medicine, Thomas Jefferson University, 1020 Locust Street, Suite 353, 19107 Philadelphia, PA, USA |
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Abstract: | Acute and chronic renal diseases remain common complications of systemic sclerosis. Although treatment for acute scleroderma
renal crisis may arrest the rapid progression of renal disease, many patients develop persistent renal dysfunction. Based
on recent insights gained from progressive renal diseases of diverse etiologies, novel approaches to understanding the pathobiology
of scleroderma renal disease may be applicable. Key factors involved in progression of renal disease include accumulation
of extracellular matrix in the glomerular and tubulointerstitial compartments, epithelial to mesenchymal transformation, and
vascular changes. The relevant factors mediating these events include the reninangiotensin system, the profibrotic growth
factors, transforming growth factor-beta and connective tissue growth factor, and reactive oxygen species. Much of the molecular
details of the role of these factors have been revealed and promise to alter the practice of therapy of progressive renal
disease. |
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Keywords: | |
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