Systemic sclerosis is a rare autoimmune, clinically heterogeneous disease. Systemic sclerosis is characterized by disturbances of the immune system, alterations of the microvasculature and by overproduction and deposition of various proteins, in particular collagen, leading to inflammation and abnormal growth of the connective tissue of the skin and internal organs such as gastrointestinal tract, lungs, heart and kidney. The disease may lead to severe dysfunction and can be life threatening due to failure of internal organs. There is at present no cure for systemic sclerosis and existing treatments, including immunosuppressive drugs, are focused on controlling symptoms and preventing complications. There is a high unmet medical need for new therapies for systemic sclerosis.