Innovative approaches in systemic sclerosis

Systemic sclerosis (SSc) is characterized by progressive fibrosis of the skin and internal organs. The chronic and highly heterogenic disease course has substantially hampered research into SSc in the past. However, the advent of new research tools and animal models has permitted a deeper insight into the pathogenesis of SSc. New findings have been described in the fields of genetic and epigenetic research, cytokine expression, pathogenic autoantibodies, and defective blood progenitor and effector cells. As a consequence, new therapeutic compounds are being developed and tested in SSc patients. Drugs that have been successfully used in other diseases are currently being investigated in controlled trials in SSc. In addition to a "targeted" approach, for example inhibiting single cytokines or protein kinases, autologous stem cell transplantation is successfully being used as a broader approach aimed at "resetting" the immune system. This article provides an overview of the most promising advances in the SSc research field, with an emphasis on pathogenic and therapeutic aspects.