Zusammenfassung
Die Nierenbeteiligung bei systemischem Lupus erythematodes (SLE), die sogenannte Lupusnephritis (LN), ist mit einer Inzidenz von etwa 40 bis 60 % eine der häufigsten Organmanifestationen. Die Nierenbeteiligung stellt nicht selten die Erstmanifestation des SLE dar bzw. tritt in den ersten 5–10 Jahren nach Diagnosestellung eines SLE auf. Zum Screening auf das Vorliegen einer LN eignet sich die Urinanalyse, bei der eine Proteinurie oder ein aktives Sediment mit Akanthozyten nachweisbar wird. Die histologische Sicherung der LN und damit der vorliegenden LN-Klasse ist zurzeit der Goldstandard der Diagnosesicherung. Zudem ist das Wissen über die LN-Klasse ein relevanter Bestandteil der adäquaten Therapieplanung bei Patienten mit SLE und LN. Hier sind insbesondere die frühe Diagnosestellung sowie das rasche Ansprechen auf die eingesetzten Therapien von prognostischer Bedeutung für den Erhalt der Nierenfunktion sowie für die Morbidität und Mortalität der zum Zeitpunkt der Erstdiagnose meist jungen Patienten. Im Mittelpunkt der Therapie stehen somit das Erreichen einer vollständigen Remission und das Vermeiden aktiver Krankheitsphasen. Aufgrund einer komplexen Pathogenese bei gleichzeitig sehr heterogenem klinischem Erscheinungsbild mit sechs verschiedenen histologischen Klassen der LN ergeben sich unterschiedliche Therapieangriffspunkte. Dies wiederum resultiert bei zunehmendem Verständnis der Signalwege und Einflussfaktoren in einer deutlichen Expansion der Studienlandschaft im Bereich der LN und erfreulicherweise in einem wachsenden Armamentarium an verfügbaren zielgerichteten Therapieoptionen. Zugleich eröffnen neue Erkenntnisse in der medikamentösen Therapie zur Progressionshemmung der chronischen Niereninsuffizienz supportive Therapieoptionen, die den Erhalt der Nierenfunktion noch verbessern können.
Abstract
Renal involvement in systemic lupus erythematosus (SLE), so-called lupus nephritis (LN), is one of the most frequent organ manifestations with an incidence of approximately 40–60%. It is not uncommon for renal involvement to be the initial manifestation of SLE or to occur in the first 5–10 years after diagnosis of SLE. Urinalysis is useful in screening for the presence of LN, demonstrating proteinuria or active sediment with acanthocytes. Histologic confirmation of LN, and thus the LN class present, is currently the gold standard for confirming the diagnosis. In addition, knowledge of the LN class is a relevant component of adequate treatment planning in SLE patients with LN. In particular, early diagnosis and rapid response to therapy are of prognostic importance for the preservation of renal function as well as morbidity and mortality of the mostly young patients at the time of initial diagnosis. Thus, the focus of therapy is to achieve complete remission, as well as to avoid active disease phases. Due to a complex pathogenesis and at the same time a very heterogeneous clinical presentation, with six different histological classes of LN, there are different therapeutic targets. This in turn results in a significant expansion of the study landscape in the field of LN with an increasing understanding of the signaling pathways and influencing factors, and fortunately in a growing armamentarium of available targeted therapy options. Simultaneously, new insights into drug therapy to inhibit progression of chronic renal disease are opening up supportive therapy options that can further improve preservation of renal function.
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J. Weinmann-Menke weist auf folgende Beziehungen hin: Vortragshonorare und Beratertätigkeit: AstraZeneca, GSK, Otsuka, Novartis, Boehringer Ingelheim, Bayer-VIfor. Studienteilnahme (klinische Studien): AstraZeneca, Boehringer-Ingelheim, Norvartis, Otsuka, Roche.
Für diesen Beitrag wurden von der Autorin keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.
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Weinmann-Menke, J. Lupusnephritis – von der Diagnose zur Therapie. Innere Medizin 64, 225–233 (2023). https://doi.org/10.1007/s00108-023-01489-y
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DOI: https://doi.org/10.1007/s00108-023-01489-y
Schlüsselwörter
- Systemischer Lupus erythematodes
- Lupusnephritis/Klassifikation
- Nierenbiopsie
- Proteinurie
- Molekular zielgerichtete Therapie