Systemic sclerosis: current views of its pathogenesis.
Systemic sclerosis (SSc) is an autoimmune disorder of unknown etiology characterized by severe and often progressive cutaneous and visceral fibrosis, pronounced alterations in the microvasculature, and numerous cellular and humoral immune abnormalities. Clinically, SSc is very heterogeneous, encompassing a spectrum ranging from mild limited forms of skin sclerosis with minimal internal organ involvement to severe skin and multiple internal organ fibrosis. Mortality and morbidity in SSc are very high and are directly related to the extent of the fibrotic and microvascular alterations. A better understanding of the pathogenesis of this incurable disorder will help to better target and design effective therapy in the future.
Derk
Chris T
CT
Department of Medicine, Division of Rheumatology, Thomas Jefferson University, Philadelphia, PA 19107, USA.
Jimenez
Sergio A
SA
D001327:Autoimmune Diseases / Q000209:etiology* / Q000235:genetics / Q000276:immunology; D002678:Chimera / Q000276:immunology; D003094:Collagen / Q000235:genetics; D005786:Gene Expression Regulation; D020022:Genetic Predisposition to Disease; D006801:Humans; D007107:Immune System / Q000276:immunology; D012595:Scleroderma, Systemic / Q000209:etiology* / Q000235:genetics / Q000276:immunology; D015398:Signal Transduction / Q000276:immunology / Q000502:physiology; D016212:Transforming Growth Factor beta / Q000378:metabolism
D016212:Transforming Growth Factor beta; D003094:Collagen