Von der renalen Krise zur Lungenfibrose – die systemische Sklerose im Wandel

Zusammenfassung

Die systemische Sklerose (SSc) ist eine Kollagenose multifaktorieller Genese, bei der autoimmune Entzündungsreaktionen zur Fibrosierung diverser Gewebe führen. In der Vergangenheit war die renale Krise eine häufige Komplikation mit sehr hoher Mortalität. Aufgrund der Empfehlung zum vorsichtigeren Einsatz von Glukokortikoiden und der Nutzung von ACE-Inhibitoren als Akutbehandlung hat sich die Inzidenz und Prognose der renalen Krise allerdings seit den 1980er-Jahren deutlich gebessert. Mittlerweile ist die Lungenbeteiligung, inklusive pulmonaler arterieller Hypertonie, interstitieller Lungenerkrankung und Lungenfibrose, die häufigste Todesursache bei SSc. Eine frühe Erkennung, auch mittels Screening-HR-CT, und die adäquate Behandlung der interstitiellen Lungenerkrankung sind daher von höchster Wichtigkeit. Mycophenolat-Mofetil (MMF) hat sich hierfür als ein wirkungsvolles Basis-Therapeutikum herausgestellt. Nintedanib ist das einzige in Deutschland zugelassene Medikament zur Therapie der progredienten Lungenfibrose bei SSc. In Studien zeigten sich die besten Prognose-Verbesserungen bei frühzeitiger Kombinationstherapie von MMF mit Nintedanib.

Abstract

Systemic sclerosis (SSc) is a connective tissue disease of multifactorial origin in which autoimmune inflammatory reactions lead to fibrosis of multiple tissues. In the past, renal crisis was a common complication with a very high mortality. Due to the recommendation for a more cautious use of corticosteroids and the use of ACE inhibitors as an acute treatment reduced the incidence of a renal crisis and improved overall survival since the 1980s. However, lung involvement including pulmonary arterial hypertension, interstitial lung disease and lung fibrosis is now the most common cause of death in SSc. An early detection, including the use of HR-CT screening, and adequate treatment of interstitial lung disease are therefore of the utmost importance. Mycophenolate mofetil (MMF) has proven to be an effective therapeutic agent for the pulmonary manifestation. Nintedanib is the only drug approved in Germany for SSc-associated progressive lung fibrosis. Studies have shown the best prognostic improvements with early combination therapy of MMF in combination with Nintedanib.

Schlüsselwörter

Systemische Sklerose - interstitielle Lungenerkrankung - Lungenfibrose - renale Krise - Nintedanib

Keywords

Systemic Sclerosis - interstitial lung disease - lung fibrosis - renal crisis - Nintedanib

Publication History

Received: 18 December 2024

Accepted after revision: 19 May 2025

Article published online:
05 August 2025

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