Systemic sclerosis (scleroderma) is characterized by autoimmune phenomena and a progressive fibrosis. Clinical signs include Raynaud's phenomenon, pathognomonic nail fold capillary changes, pulmonal, renal, cardial and intestinal involvement. The two forms of systemic sclerosis, the diffuse and the limited show distinct disease phenomena and evolution. The diffuse form typically leads to a rapid sclerosis of the whole skin, it is associated with the auto-antibody Scl-70, it presents with an interstitial fibrosis of the lung, myositis and cardial fibrosis, a progressive loss of intestinal motility with maldigestion and it bears the risk of renal crisis. The limited form is characterized by sclerosis of the skin of the distal parts of the limbs and the face only, it is associated with the centromer auto-antibody and a much slower progression of visceral fibrosis. A typical late stage manifestation is pulmonary arterial hypertension. New drugs have improved quality of life. Their correct indication is based on a systematic monitoring of disease.