Progressive systemic sclerosis-from the molecular background to innovative therapies

Abstract

Systemic sclerosis (SSc) is a complex autoimmune disorder. The cornerstones of the pathogenesis are vascular damage, fibrogenesis and various cellular and humoral autoimmune processes. The aim of the present review is to describe pathogenic steps, leading to the hallmark clinical picture of SSc. Indeed, numerous therapeutical approaches have been tested/are in use, directed towards vascular damage, fibrogenesis, as well as autoimmune processes in order to decelerate the progression of the disease. These therapies are also discussed in the review. Finally, we described certain novel immune-modulating possibilities, namely autologous stemcell transplantation and extracorporeal photochemotherapy.