Systemic Sclerosis: From Pathogenesis Towards Targeted Immunotherapies

Systemic sclerosis (SSc), a chronic disease with widespread collagen deposition, has three pathogenetic facets: immune activation, microvasculopathy and fibroblast activation. Immune activation and microvasculopathy occur very early in the disease process, and inflammatory infiltrates in the skin are restricted in early-phase disease. There is good evidence that fibroblast activation with collagen production may be triggered by the immune system. In early-phase disease, we slowly move from general immunosuppression to therapeutically targeting specific molecules involved in immune activation, such as T cell-directed targets, B cell-directed targets, cytokine targets, and tyrosine kinases targets.