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The pathogenesis of fibrosis and renal disease in scleroderma: Recent insights from glomerulosclerosis

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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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Authors and Affiliations

  1. Dorrance Hamilton Research Laboratories, Division of Nephrology, Department of Medicine, Thomas Jefferson University, 1020 Locust Street, Suite 353, 19107, Philadelphia, PA, USA

    Kumar Sharma MD

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  1. Sungchun Lee MD
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  2. Sohee Lee MD
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  3. Kumar Sharma MD
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Lee, S., Lee, S. & Sharma, K. The pathogenesis of fibrosis and renal disease in scleroderma: Recent insights from glomerulosclerosis. Curr Rheumatol Rep 6, 141–148 (2004). https://doi.org/10.1007/s11926-004-0059-3

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