Zusammenfassung
Die systemische Sklerodermie ist eine seltene, mehrere Organsysteme betreffende Autoimmunerkrankung unklarer Genese. Der Krankheitsverlauf ist mit den heutigen therapeutischen Möglichkeiten schwer zu kontrollieren. Iloprost, ein stabiles Prostazyklinanalogon, kann durch seinen antifibrotischen Effekt einen wichtigen Schritt in der Pathogenese der Sklerodermie beeinflussen. In dieser Übersichtsarbeit wurde aus der Literatur die optimale Dosierung und Therapiedauer herausgearbeitet. In 3 verschiedenen Studien erwies sich eine Infusionstherapie über 5 Tage in einer vom Patienten am besten vertragenen Maximaldosis (1–2 ng/kg/min) als wirksam mit einem signifikanten Effekt auf die Hautveränderungen wie Schwere und Zahl der Raynaud-Anfälle, die Heilung der Ulzera und die digitale Durchblutung. Diese Wirkung hält etwa 4 Wochen an und kann durch Wiederholung der Infusionen 1-mal/Monat aufrechterhalten werden. Obwohl noch weitere Studien erforderlich sind, um einen Langzeiteffekt zu überprüfen, dürfte eine intermittierende Dauertherapie mit Iloprost zu einer Verbesserung oder Stabilisierung der Hautveränderungen bei Sklerodermie führen.
Abstract
Systemic sclerosis is an autoimmune disease of unknown origin affecting multiple organ systems. The management of this disease is challenging. Many therapeutic attempts have only been moderately successful. Iloprost, a stable prostacyclin analogue, with its antifibrotic effect can influence an important step in the pathogenesis of systemic sclerosis. In this review we analyze the published data for the optimal dose and duration of treatment. In three different studies, iloprost given for 5 days in the highest tolerated dose of 1–2 ng/kg/min provided a significant improvement of the disease measured by the number and intensity of Raynaud attacks, healing of digital ulcers, and digital perfusion. This improvement lasted for about one month. When the infusions were repeated once a month, treatment effect could be maintained. Although the effect of this treatment regimen should be proven in further long-term studies, we think that an intermittent continuous therapy with iloprost could result in an improvement or stabilization of systemic sclerosis.
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Bali, G., Aberer, E. Iloprosttherapie bei systemischer Sklerodermie. Hautarzt 54, 845–851 (2003). https://doi.org/10.1007/s00105-003-0578-2
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DOI: https://doi.org/10.1007/s00105-003-0578-2