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KMID : 0604220070140030073
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Korean Journal Investigative Dermatology
2007 Volume.14 No. 3 p.73 ~ p.80
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Pathogenesis of Scleroderma
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Cho So-Yun
Chung Jin-Ho
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Abstract
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Scleroderma is a heterogeneous group of disorders in which vascular and immunological disturbances lead to tissue fibrosis. Systemic sclerosis and limited cutaneous forms share similar pathogenesis: vascular endothelial damage followed by upregulation of adhesion molecules and chemotactic cytokines causes inflammatory cell recruitment and infiltration through endothelium to extracellular matrix. Subsequent activation of TGF-β pathway and other factors including CTGF, IL-1, IL-4, IL-6, endothelin-1 and TIMP-1 leads to activation of fibroblasts and overproduction of collagen. Endothelin-1 and PDGF cause transdifferentiation of vascular smooth muscle cells to myofibroblasts, which leads to narrowing of vessel lumen.
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KEYWORD
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Scleroderma, Pathogenesis, TGF-β, Collagen
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