Systemische Sklerose

Abstract

Systemic sclerosis is one of the most severe rheumatic diseases with a strong impact on quality of life and prognosis. Interstitial lung disease, pulmonary arterial hypertension (PAH) and cardiac manifestations are the most important reasons for death. Pain, loss of range of motion, and digital ulcers often determine quality of life. There is a high prevalence of malnutrition, sarcopenia, depression, osteoporosis, cardiovascular events, and malignancies. Chronic activation of the immune system might contribute to the increased rate of these co-morbidities. We have some initial approaches to understand the mechanisms of the disease and the link between obliterative vasculopathy and the variable existence of fibrosis. Treatment should start early. Several drugs have shown efficacy to improve Raynaud's phenomenon and obliterative vasculopathy. Unfortunately, they are often used inconsistently. Treatments such as cyclophosphamide, mycophenolate mofetil, or autologous stem cell transplantation have shown to improve inflammatory fibrosis in clinical studies of systemic sclerosis. New drugs targeting key mechanisms could provide novel approaches with fewer side effects. Several studies are ongoing. Consistent individual and target-oriented therapies are the key to a successful treatment of patients with systemic sclerosis.