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Therapie des steroidsensiblen nephrotischen Syndroms

Treatment of steroid-sensitive nephrotic syndrome

  • Leitthema
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Zusammenfassung

Die Inzidenz des steroidsensiblen nephrotischen Syndroms (SSNS) in Deutschland beträgt 1,2:100.000 Kinder und Jugendliche unter 18 Jahren. Primär steroidsensibel sind 85% aller Patienten mit idiopathischem nephrotischem Syndrom (INS). Die Standardtherapie erfolgt mit Prednison über 6 Wochen und anschließender alternierender Gabe alle 2 Tage für weitere 6 Wochen. Auch zur Standardrezidivtherapie wird Prednison verwendet. Die Zweitlinientherapie sollte mit dem risikoärmsten Medikament Levamisol beginnen. Über den Einsatz von Cyclophosphamid (CP) muss individuell entschieden werden. Ciclosporin A (CSA) ist sehr effektiv, doch man tauscht die Steroid- gegen eine CSA-Abhängigkeit ein, zudem geht die CSA-Dauertherapie häufig mit Nebenwirkungen einher. Mycophenolatmofetil (MMF) entwickelt sich zu einer Therapieoption der Zukunft, doch ist es für die Behandlung des INS nicht zugelassen. Alle weiteren Therapieoptionen entbehren den Beweis der Wirksamkeit oder sind ihn noch schuldig. Einige Patienten bedürfen einer langfristigen intensiven Therapie und Überwachung, dennoch gilt angesichts der guten Prognose des SSNS der ärztliche Grundsatz in besonderem Maße: primum nihil nocere.

Abstract

The incidence of steroid-sensitive nephrotic syndrome (SSNS) in Germany is 1.2/100,000 individuals under the age of 18 years, and 85% of all patients with idiopathic nephrotic syndrome are steroid-sensitive. The standard therapy is prednisone for 12 weeks; relapses are also treated by prednisone. Second-line medication starts with levamisole because of its lower risk of side effects. Cyclophosphamide is effective in SSNS, but its adverse effects are evident. Cyclosporine works well, but it replaces steroid dependency with cyclosporine dependency and carries a risk of many side effects. Mycophenolate mofetil appears to be a promising alternative but still lacks official endorsement. The other options have no effect on relapse rate or need more investigation. Some patients need long-term immunosuppressive therapy and monitoring. In light of the good prognosis for patients with SSNS, it is essential to follow the basic medical rule: primum nihil nocere.

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  1. Pädiatrische Nephrologie, Abteilung für Allgemeine Kinderheilkunde und Poliklinik, Zentrum für Kinderheilkunde, Universitätsklinikum Bonn, Adenauerallee 119, 53113, Bonn, Deutschland

    I. Franke

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Franke, I. Therapie des steroidsensiblen nephrotischen Syndroms. Monatsschr Kinderheilkd 157, 226–234 (2009). https://doi.org/10.1007/s00112-008-1867-8

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