[Systemic sclerosis].
Systemic sclerosis (SSc), the focus of this review, is a generalized connective tissue disease that involves sclerotic changes in the skin and sometimes various other organ systems. Clinical outcomes have improved probably due to better management of the complications, but SSc is still considered to be incurable and diffuse cutaneous SSc carries high risk of fatality. Although the pathogenesis of SSc is still unknown, the basic mechanism appears to involve endothelial cell injury, overproduction of extracellular matrix (ECM), and aberrant immune activation. This review discusses recent studies that investigated the cellular and molecular mechanisms in the pathogenesis of SSc.
Ihn
Hironobu
H
Graduate School of Medical and Pharmaceutical Sciences, Kumamoto University.
D000974:Antibodies, Antinuclear; D003094:Collagen / Q000235:genetics; D042783:Endothelial Cells / Q000473:pathology; D019332:Endothelin-1 / Q000502:physiology; D005109:Extracellular Matrix / Q000378:metabolism; D005347:Fibroblasts / Q000502:physiology; D006801:Humans; D008214:Lymphocytes / Q000276:immunology; D010982:Platelet-Derived Growth Factor / Q000276:immunology / Q000502:physiology; D012595:Scleroderma, Systemic / Q000209:etiology / Q000473:pathology; D014157:Transcription Factors / Q000502:physiology; D016212:Transforming Growth Factor beta / Q000502:physiology
D000974:Antibodies, Antinuclear; D019332:Endothelin-1; D010982:Platelet-Derived Growth Factor; D014157:Transcription Factors; D016212:Transforming Growth Factor beta; D003094:Collagen