Systemic Sclerosis

Systemic sclerosis (SSc) is a rare and severe autoimmune disease characterized by thickening of the skin caused by accumulation of collagen. The pathophysiology of SSc is incompletely understood but excessive fibrosis of connective tissue, vasculopathy and immune components are involved. Skin and other organs, including the kidneys, heart, lungs, and gastrointestinal tract, may be affected. There is at present no targeted disease modifying treatment for SSc. Current treatments aim to relieve the symptoms, and in some instances immune dampening medications as well as vascular dilatory treatments can be given. Thus, there is a major unmet medical need, which would benefit from a better understanding of the cellular mechanisms underlying the disease.