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.
Keywords: B cell, fibroblast, immune activation, immunotherapy, systemic sclerosis, targeted therapy, T cell
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