Systemische Sklerose

Systemic sclerosis (SSc) is a non-curable autoimmune disease with a high impact on patients' quality of life and morbidity. SSc is a connective tissue disease characterised by microangiopathy, production of autoantibodies and dysfunction of fibroblasts leading to increased production of extracellular matrix. In addition to skin affection with acral ulcerations and autoamputations, there may be an involvement of lungs, heart, gastrointestinal tract, and kidneys. Main causes of death are lung fibrosis, pulmonary arterial hypertension, and cardiac manifestations. There is an association with specific anti-nuclear antibodies; the pathogenesis of the disease remains enigmatic until today. Some initial attempts have been made to understand disease mechanisms and relations between obliterative vasculopathy and fibrosis. Treatment should start as soon as possible and is a combination of immunosuppressants, antifibrotic, and vasodilative therapies depending on the organ involvement.