Abstract

Systemic sclerosis and its subtypes differ significantly from other diseases in rheumatology and clinical immunology, as the aberrant activation of the immune system does not result in an inflammation-driven destruction but in a progressive matrix synthesis, especially of the skin. Owing to the recently established networks in Germany (DNSS) as well as in Europe (EUSTAR), a detailed profile of the affected patients could be determined. No specific predictive markers for the disease exist in the early phases of the disease although a Raynaud phenomenon can be present up to ten years prior to the first organ manifestations. On the other hand, distinct clinical features and laboratory parameters can be independent predictive markers for the outcome of a given patient. Therefore, monitoring should be performed on a regular basis in the preclinical and early stages of the disease, as early diagnosis can reduce morbidity and mortality of the affected patients significantly, especially with regard to pulmonary hypertension, digital ischemia, reflux esophagitis and sclerodermal renal crisis.