Systemische Sklerose

Zusammenfassung

Die systemische Sklerose gehört zu den rheumatischen Erkrankungen mit dem höchsten Einfluss auf die Prognose und Lebensqualität. Organmanifestationen wie die interstitielle Lungenfibrose, die pulmonale arterielle Hypertonie (PAH) und die Herzbeteiligung bestimmen die Mortalität, während Schmerzen, Bewegungseinschränkungen und digitale Ulzerationen häufig die Lebensqualität beeinflussen. Mangelernährung, Sarkopenie, Depression, Osteoporose, kardiovaskuläre Erkrankungen und Tumorerkrankungen treten möglicherweise bedingt durch die chronische Aktivierung des Immunsystems gehäuft auf. Es gibt derzeit erste Ansätze, Krankheitsmechanismen und die Beziehung zwischen der obliterativen Vaskulopathie und der variabel vorhandenen Fibrose zu verstehen. Die Therapie sollte frühzeitig beginnen. Eine Reihe von Substanzen beeinflusst die Raynaud-Symptomatik und die obliterative Gefäßerkrankung. Leider werden diese Therapien oftmals nicht ausreichend genutzt. Immunsuppressiva wie Cyclophosphamid, MMF oder eine autologe Stammzelltransplantation haben zu einer deutlichen Verbesserung der inflammatorischen Fibrose in klinischen Studien geführt. Neue Therapieansätze, die Krankheitsmechanismen beeinflussen und nebenwirkungsärmer sind, erscheinen erfolgversprechend und könnten künftig zu einer Zulassung führen. Derzeit wird eine Vielzahl von Studien durchgeführt. Die konsequente zielorientierte und individuelle Therapie stellt einen wichtigen Schlüssel zum Therapieerfolg dar.

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.

Schlüsselwörter

Systemische Sklerose - Lungenfibrose - Autoantikörper - vasoaktive Therapie - Biologika

Key words

systemic sclerosis - vasoactive therapy - autoantibody - biologicals - pulmonary fibrosis

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