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Implementation von Leitlinien für eine Therapie der systemischen Sklerose (Sklerodermie)

Wunsch und Wirklichkeit

Towards an implementation of guidelines for the therapy of systemic sclerosis (scleroderma)

Between desire and reality

Zeitschrift für Rheumatologie Aims and scope Submit manuscript

Zusammenfassung

Drei Charakteristika kennzeichnen die klinischen Manifestationen der systemischen Sklerose (Sklerodermie, SSc): Autoimmunität/Entzündung, Gefäßschädigung (Vaskulopathie) sowie (Organ-) Fibrose. Inzwischen sind medikamentöse und andere Therapieansätze für jedes dieser Charakteristika verfügbar. Aufgrund der Seltenheit der Erkrankung und ihrer klinischen Heterogenität fehlen jedoch oftmals gute Therapiestudien. Eine Initiative der „EULAR Scleroderma Trials and Research Group“ (EUSTAR, eine Arbeitsgruppe innerhalb der „European League Against Rheumatism“, EULAR) hat deshalb begonnen, die bisher verfügbaren Therapien zu bewerten und Empfehlungen auszusprechen. In diesem Artikel werden diejenigen Therapieansätze diskutiert, die über diese Empfehlungen hinaus zur Behandlung der systemischen Sklerose eingesetzt werden können. Es ist zu erwarten, dass durch die Etablierung von nationalen und internationalen Netzwerken (z. B. Deutsches Netzwerk für Systemische Sklerodermie, DNSS, oder EUSTAR) über die Auswertung großer Registerdaten und Durchführung gezielter Therapiestudien die Evidenz zur Behandlung für die systemische Sklerose zukünftig verbessert werden kann.

Abstract

The clinical manifestations of systemic sclerosis (scleroderma, SSc) are characterized by three prominent features: autoimmunity/inflammation, vascular lesions (vasculopathy) and (organ) fibrosis. Drugs and other therapies are now available for each of these features. However, due to the low prevalence and high variation in clinical signs and symptoms of the disease, there are only few high quality clinical trials. The EULAR Scleroderma Trials and Research Group (EUSTAR, a subgroup of the European League Against Rheumatism, EULAR) has therefore started to assess the therapies available today and make recommendations. The present article discusses treatment options in systemic sclerosis beyond these recommendations. It is to be expected that the establishment of national and international networks for systemic sclerosis research (e.g. the German Network for Systemic Sclerosis, DNSS, and EUSTAR) will raise the standards of evidence-based therapy for systemic sclerosis in the future by analyzing large data sets and performing clinical trials.

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Becker, M., Müller-Ladner, U. & Riemekasten, G. Implementation von Leitlinien für eine Therapie der systemischen Sklerose (Sklerodermie). Z. Rheumatol. 69, 310–317 (2010). https://doi.org/10.1007/s00393-009-0523-6

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