Systemische Sklerose

Zusammenfassung

Die Systemische Sklerose (Synonym: systemische Sklerodermie) ist eine nicht heilbare Autoimmunerkrankung mit einem hohen Einfluss auf die Lebensqualität und die Morbidität der Patienten. Sie zählt zu den Kollagenosen und ist gekennzeichnet durch eine generalisierte Mikroangiopathie, die Produktion von Autoantikörpern und durch Dysfunktion der Fibroblasten, die zu erhöhter Produktion von extrazellulärer Matrix führt. Neben der Hautbeteiligung mit akralen Ulzerationen und Autoamputationen kann eine Beteiligung der inneren Organe wie Lunge, Herz, Gastrointestinaltrakt und Niere vorkommen. Die Haupttodesursachen stellen die interstitielle Lungenerkrankung, die pulmonal-arterielle Hypertonie und die kardiale Manifestation dar. Es besteht eine Assoziation mit bestimmten antinukleären Antikörpern, die Pathogenese ist bis heute noch weitestgehend unverstanden. Es gibt derzeit erste Ansätze, Krankheitsmechanismen und die Beziehung zwischen der obliterativen Vaskulopathie und der Fibrose zu verstehen. Die Therapie sollte so frühzeitig wie möglich im Krankheitsverlauf beginnen und ist je nach Organbefall eine Kombination aus Immunsuppressiva, antifibrotischer oder vasodilatativer Therapie.

Abstract

Systemic sclerosis (SSc) is a non-curable autoimmune disease with a high impact on patients’ quality of life and morbidity. SSc is a connective tissue disease characterised by microangiopathy, production of autoantibodies and dysfunction of fibroblasts leading to increased production of extracellular matrix. In addition to skin affection with acral ulcerations and autoamputations, there may be an involvement of lungs, heart, gastrointestinal tract, and kidneys. Main causes of death are lung fibrosis, pulmonary arterial hypertension, and cardiac manifestations. There is an association with specific anti-nuclear antibodies; the pathogenesis of the disease remains enigmatic until today. Some initial attempts have been made to understand disease mechanisms and relations between obliterative vasculopathy and fibrosis. Treatment should start as soon as possible and is a combination of immunosuppressants, antifibrotic, and vasodilative therapies depending on the organ involvement.

Schlüsselwörter

Systemische Sklerose - Kollagenose - Autoimmunerkrankung

Keywords

systemic sclerosis - connective tissue disease - autoimmune disease

Publication History

Article published online:
26 June 2024

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