SISTEMSKA SKLEROZA – UVODNO PREDAVANJE

Dušanka Martinović Kaliterna, Mislav Radić

The pathogenesis of systemic sclerosis (SSc) is complex and appears to involve endothelium, epithelium, fibroblasts and immunological mediators, resulting in dysregulated vascular remodelling and ultimately vasculopathy. Endothelial cell injury is an early and probably initiating event, but the precise aethyology remains unclear. Vascular damage and tissue fibrosis are widespread in SSc and largely account for the protean clinical manifestations and substantial morbidity and mortality. Current therapies are primarily immunomodulatory in nature. Effective therapy will require directly targeting the fibrotic process and will necessitate an improved understanding of the roles of individual cell types and their products in the development of fibrosis.

Sistemska skleroza (SSc) je patogenetski složena bolest. Drži se da su u zbivanje uključeni endotel, epitel, fibroblasti i imunološki medijatori, rezultat je poremećeno vaskularno remodeliranje i u konačnici vaskulopatija. Endotelna stanična ozljeda je rani i vjerojatno početni događaj, iako etiološka zbivanja nisu posve jasna. Vaskularno oštećenje i tkivna fibroza su prošireni u SSc i glavna su podloga kliničkih manifestacija, te posljedičnog morbiditeta i mortaliteta. Današnja terapija SSc je u naravi imunomodulatorna. Učinkovita terapija podrazumijeva direktno uplitanje u fibrozirajuće procese, što zahtijeva točnu spoznaju o ulozi pojedinih stanica i njihovih produkata u nastanku fibroze.