Systemische Sklerose

Zusammenfassung

Die systemische Sklerose stellt aufgrund ihrer zahlreichen Organmanifestation und ihrem Einfluss auf die Lebensqualität und Morbidität eine große Herausforderung dar. Lungenfibrose, pulmonale arterielle Hypertonie (PAH) und kardiale Manifestationen bestimmen die krankheitsbezogene Mortalität. Die Patienten weisen zudem eine Vielzahl von Komorbiditäten wie Mangelernährung, Depression, Osteoporose und ein erhöhtes Risiko für kardiovaskuläre Erkrankungen auf, welche erkannt und therapiert werden sollten. Das frühzeitige Erfassen und die Quantifizierung von Schäden sind neben der frühen und intensiven Therapie ein Schlüssel für den Therapieerfolg. Die Entdeckung von pathogenetisch bedeutsamen Autoantikörpern und die Kenntnis, dass intensive immunsuppressive und vasoaktive Therapien erfolgreich sind, sind wesentliche Fortschritte. Derzeit werden verschiedene Forschungsansätze überprüft. Therapien mit Biologika erscheinen erfolgversprechend und könnten künftig zur Zulassung führen.

Abstract

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.

Schlüsselwörter

Systemische Sklerose - Lungenfibrose - Angiotensin-Rezeptor-Autoantikörper - Endothelin-Rezeptor-Autoantikörper - Biologika - vasoaktive Therapie - Sklerodermie

Keywords

scleroderma - systemic sclerosis - lung fibrosis - vasoactive therapy - biologicals - angiotensin 1 receptor antibody - endothelin receptor antibody

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