title: Pathophysiology of systemic sclerosis: state of the art in 2014.
abstract: Major work has been done in order to improve the understanding of systemic sclerosis (SSc) pathogenesis. A number of new experimental models have been set up, that should help to understand the disease pathogenesis and test new therapeutic targets. Reactive oxygen species represent a hallmark of the pathogenesis of SSc, both at the fibroblast and at the endothelial cell levels. Although a large number of genetic studies have been conducted, it is still difficult to identify a genetic background specific to SSc, and the major progress in this setting is probably the identification of an interferon signature. Besides endothelial cells and fibroblasts, major development has been made in the understanding of the role of B cells and autoantibodies in the pathogenesis of SSc. Plasmacytoid dendritic cells seem to play a major role in the pathogenesis of SSc through the secretion of CXCL4, although these data will need to be confirmed in the near future.
authors.affiliation: Institut Cochin, Inserm U1016, CNRS UMR 8104, 8, rue Méchain, 75014 Paris, France; Université Paris Diderot, 75013 Paris, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Dumoitier
authors.affiliation: Institut Cochin, Inserm U1016, CNRS UMR 8104, 8, rue Méchain, 75014 Paris, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Lofek
authors.affiliation: Institut Cochin, Inserm U1016, CNRS UMR 8104, 8, rue Méchain, 75014 Paris, France; Assistance publique-Hôpitaux de Paris (AP-HP), hôpital Cochin, centre de référence pour les vascularites nécrosantes et la sclérodermie systémique, service de médecine interne, Université Paris Descartes, 75014 Paris, France. Electronic address: luc.mouthon@cch.aphp.fr.
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D005109:Extracellular Matrix; D005347:Fibroblasts / Q000502:physiology; D006801:Humans; D007107:Immune System / Q000502:physiology; D012595:Scleroderma, Systemic / Q000235:genetics / Q000276:immunology / Q000503:physiopathology*