[Pathogenesis of systemic sclerosis].
Systemic sclerosis is a complex multi-systemic disease with a mostly unresolved pathogenesis. Following an inflammatory reaction, overproduction of collagen and other extra-cellular matrix components leads to a characteristic fibrosis. It remains unclear why this overproduction by fibroblasts and myofibroblasts occurs. Micro-vascular disturbances and endothelial cells, as well as immunomodulation and inflammation are central factors. Besides intrinsic influences, such as genetic polymorphisms, multiple mediators with fibrotic effects such as Platelet Derived Growth Factor, Transforming Growth Factor-beta and Connective Tissue Growth Factor have been characterized. These have become targets for innovative therapeutic strategies that might lead to specific treatments for systemic sclerosis.
Fabri
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M
Klinik und Poliklinik für Dermatologie und Venerologie, Universität zu Köln, Kerpenerstrasse 62, 50924, Köln, Germany.
Krieg
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T
D000818:Animals; D000974:Antibodies, Antinuclear / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D003095:Collagen Diseases / Q000503:physiopathology; D004195:Disease Models, Animal; D042783:Endothelial Cells / Q000502:physiology; D005109:Extracellular Matrix / Q000502:physiology; D005347:Fibroblasts / Q000502:physiology; D005355:Fibrosis; D006801:Humans; D051379:Mice; D010982:Platelet-Derived Growth Factor / Q000502:physiology; D012595:Scleroderma, Systemic / Q000503:physiopathology*; D012867:Skin / Q000503:physiopathology; D016212:Transforming Growth Factor beta / Q000502:physiology; D014661:Vasoconstriction / Q000502:physiology