Systemische Sklerose

Systemic sclerosis is a challenging and heterogeneous disease due to the involvement of multiple organs and the high impact on morbidity and quality of life. Lung fibrosis, pulmonary arterial hypertension, and cardiac manifestations are main causes of systemic sclerosis-related deaths. In addition, patients suffer from a various range of co-morbidities such as malnutrition, depression, osteoporosis, malignancies, which are increased in these patients and have to be identified and treated. Early assessment of organ damage is a key to therapeutic success. The discovery of pathogenic autoantibodies combined with increased evidence of effective immunosuppressive and vasoactive treatment strategies are major developments in the therapy of the disease. At present, several clinical studies are ongoing and some of the biological therapies are promising.