[Systemic sclerosis--a challenge in rheumatology]

The connective tissue disease systemic sclerosis (SSc) is still a challenge to every rheumatologist. SSc is characterized by progressing fibrosis of the skin and internal organs, abnormal activation of the immune system and distinct changes in microcirculation. Although it is a rare disease with a prevalence of about 20:100,000 one may come across it in daily practice. SSc is classified into limited and diffuse forms. Both entities usually involve internal organs. Life expectancy is limited and depends predominantly on the extent of the organs involved. Therefore, it is essential to diagnose SSc early and to identify and closely monitor the organs involved.