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Nephrotisches Syndrom des Kindes- und Jugendalters

Zusammenfassung der AWMF-Leitlinie unter Berücksichtigung besonderer Aspekte der Diagnostik und Therapie bei Erwachsenen

Nephrotic syndrome in childhood and adolescence

Summary of the AWMF guidelines considering special aspects of diagnostics and treatment in adults

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Der Nephrologe Aims and scope

Zusammenfassung

Hintergrund

Das nephrotische Syndrom (NS) im Kindesalter erfüllt die Kriterien einer seltenen Erkrankung, und doch ist es die häufigste Glomerulopathie in dieser Altersgruppe. Gleichzeitig ist es eine heterogene Erkrankung, die durch die Kategorien Alter bei Erstmanifestation, Ätiologie, Histologie und Ansprechen auf eine Standardtherapie mit Glukokortikoiden eingeteilt werden kann. Das primäre Ansprechen auf eine Standardtherapie mit Glukokortikoiden differenziert zwischen steroidsensiblem (SSNS) und steroidresistentem NS (SRNS) und ist der wichtigste Prognosefaktor. Eine hohe kumulative Dosis an Glukokortikoiden (Steroiden) oder die verlängerte Gabe bei Behandlung der Erstmanifestation beeinflussen das Rezidivrisiko nicht.

Eine molekulargenetische Abklärung bei Patienten mit SRNS ist zur Vermeidung einer wirkungslosen immunsuppressiven Therapie dringend zu empfehlen.

Methoden

Der vorliegende Text gibt eine Übersicht zur Therapie des NS, beleuchtet Unterschiede in der Diagnostik und Therapie im Kindes- und Erwachsenenalter und beschreibt Risiken und Langzeitfolgen der Therapie.

Schlussfolgerung

Häufige Rezidive des NS und das SRNS stellen weiterhin eine therapeutische Herausforderung dar. Der Einsatz von Steroiden und einer immunsuppressiven Erhaltungstherapie hat erhebliche Langzeitauswirkungen. Die optimale Behandlung von Patienten mit SSNS mit seltenen Rezidiven im Erwachsenenalter ist unklar.

Abstract

Background

Despite fulfilling the criteria for an orphan disease, idiopathic nephrotic syndrome (NS) in children is the most frequent glomerular disease in this age group. At the same time NS in children is a heterogeneous disease and in order to assess the individual facets of the disease a classification using the following criteria is helpful: etiology, age at onset, histology and responsiveness to initial standard treatment with glucocorticoids. As for prognosis the differentiation between steroid-sensitive (SSNS) and steroid-resistant nephrotic syndromes (SRNS) is most important because SRNS is a risk factor for developing end-stage renal disease. High cumulative doses of glucocorticoids or prolonged administration in the initial treatment do not influence the risk of a relapse. Genetic testing is highly advisable in patients with SRNS to avoid unnecessary and ineffective immunosuppressive therapy.

Methods

This manuscript gives an overview on the management of SSNS and SRNS and provides insights into the different diagnostic and therapeutic approaches in pediatric and adult medicine. Furthermore, associated risks and potential long-term side effects of treatment are described.

Conclusion

Frequently relapsing NS and SRNS are still challenging to treat. The use of steroids and immunosuppressive maintenance therapy has severe long-term side effects. The optimal treatment of adult patients with childhood onset SSNS and infrequent relapses remains unclear.

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Danksagung

Wir möchten uns bei den weiteren Autoren der AWMF-Leitlinie für ihre Mitarbeit bedanken: Jörg Dötsch, Jutta Gellermann, Peter F. Hoyer, Marcus J. Kemper, Kay Latta, Burkhard Tönshoff und Wolfgang Rascher.

Förderung

R. Ehren und L.T. Weber werden durch die KFO 329 „Disease pathways in podocyte injury – from molecular mechanisms to individualized treatment options“ der DFG sowie durch das BMBF im Rahmen der INTENT-Studie gefördert. P. Brinkkötter wird durch die KFO 329 der DFG gefördert.

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Authors and Affiliations

  1. Abteilung für Pädiatrische Nephrologie, Immunologie und Hypertensiologie, Klinik und Poliklinik für Kinder- und Jugendmedizin, Uniklinik Köln, Kerpener Str. 62, 50937, Köln, Deutschland

    R. Ehren & L. T. Weber

  2. Klinik II für Innere Medizin, Uniklinik Köln, Köln, Deutschland

    P. Brinkkötter

  3. Klinik für Pädiatrie mit Schwerpunkt Gastroenterologie, Nephrologie und Stoffwechselmedizin, Charité Universitätsmedizin Berlin, Berlin, Deutschland

    U. Querfeld

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  1. R. Ehren
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Correspondence to R. Ehren.

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Interessenkonflikt

R. Ehren, P. Brinkkötter, L.T. Weber und U. Querfeld geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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B. Tönshoff, Heidelberg

L.T. Weber, Köln

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Ehren, R., Brinkkötter, P., Weber, L.T. et al. Nephrotisches Syndrom des Kindes- und Jugendalters. Nephrologe 14, 184–191 (2019). https://doi.org/10.1007/s11560-019-0313-3

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