Zusammenfassung
Steroidabhängiges und steroidresistentes nephrotisches Syndrom bleiben eine therapeutische Herausforderung, da Steroide und die alternativ/additiv verwendeten Immunsuppressiva ein sehr ungünstiges Nebenwirkungsprofil aufweisen. Rituximab (RTX) konnte bei Kindern mit steroidabhängigem nephrotischen Syndrom bei primärer fokal-segmentaler Glomerulosklerose (pFSGS) und Minimal-Change-Disease (MCD) bereits erfolgreich eingesetzt werden. Bei erwachsenen Patienten mit pFSGS oder MCD gibt es einzelne Fallberichte und Fallserien, welche keine einheitlichen Ergebnisse zeigen, denenzufolge aber Patienten mit steroidabhängigem nephrotischen Syndrom und vor allem Patienten mit MCD am meisten von einer RTX-Therapie profitieren dürften. Um die Wertigkeit der Therapie zu sichern, sind aber weitere kontrollierte und randomisierte Studien notwendig, die insbesondere RTX mit der Standardtherapie vergleichen.
Abstract
Steroid-resistant and steroid-dependent nephrotic syndromes remain a therapeutic challenge as long-term steroid use and alternatively/additionally used immunosuppressive agents exhibit an extremely unfavorable spectrum of adverse events. Rituximab (RTX) has emerged as an efficacious therapy for childhood steroid-dependent nephrotic syndrome due to primary focal segmental glomerulosclerosis (pFSGS) and minimal change disease (MCD). In adult patients with pFSGS and MCD single case reports and case series have shown variable therapeutic response rates after RTX therapy. In general, patients with steroid-dependent nephrotic syndrome and in particular patients with MCD have shown an impressive clinical improvement after RTX therapy. To evaluate the efficacy of RTX randomized controlled trials in a prospective manner would be highly desirable.
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Kronbichler, A., Rudnicki, M. & Mayer, G. Rituximab bei primärer FSGS und MCD im Erwachsenenalter. Nephrologe 8, 144–149 (2013). https://doi.org/10.1007/s11560-012-0689-9
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DOI: https://doi.org/10.1007/s11560-012-0689-9