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„State-of-the-art“: C3-Glomerulopathie und membranoproliferative Glomerulonephritis

Ein Konsensus

State of the art: C3 glomerulopathy and membranoproliferative glomerulonephritis

A consensus

  • CME Zertifizierte Fortbildung
  • Published:
Der Nephrologe Aims and scope

Zusammenfassung

Basierend auf neuen pathophysiologischen Erkenntnissen der vorausgehenden Jahre, erfolgte im Jahr 2010 die Einführung einer neuen Klassifikation einer Glomerulonephritis mit dominanten oder kodominanten C3-Ablagerungen, deren wesentliche Untergruppe die C3-Glomerulopathie (C3G) darstellt. Während sekundäre Ursachen zu einer immunkomplexvermittelten membranoproliferativen Glomerulonephritis (MPGN) führen, liegen die Hauptursachen der C3-Glomerulopathie primär in Störungen des Komplementsystems, die dann häufig in Form einer MPGN, aber auch anderer lichtmikroskopischer Erscheinungsbilder apparent werden. Die Kenntnis der zugrunde liegenden Pathophysiologie ist für die weiterführende Diagnostik zur Abklärung sekundärer Ursachen wegweisend. Eine umfassende Komplementanalytik, begleitet von Antikörperscreening und humangenetischer Analyse, sollte konsequent erfolgen. Wenn auch bis heute durch Studien nicht systematisch validiert, bieten die vorliegenden Erkenntnisse doch eine robuste Grundlage für die Anwendung verfügbarer Therapieansätze für diese häufig rasch progredienten und nach Nierentransplantation oftmals wiederkehrenden Krankheitsbilder. In der vorliegenden Übersicht werden die derzeitigen Erkenntnisse systematisch dargestellt und in Form eines (nationalen) Expertenkonsensus zur Diagnostik der C3-dominanten Glomerulonephritis und der MPGN zusammengefasst.

Abstract

Based on new pathophysiological discoveries in recent years, a new classification of glomerulonephritis with dominant or codominant C3 deposits was introduced in 2010, which represents the essential subgroup of C3 glomerulopathy (C3G). Although secondary causes can lead to immune complex-mediated membranoproliferative glomerulonephritis (MPGN), the major trigger of C3G is primarily dysregulation of the alternative complement pathway, which can often become apparent in the form of MPGN as well as various light microscopic phenotypes. Knowledge of the underlying pathophysiology of the disease is of high relevance for a subsequent differentiation after assessment of secondary causes. Initiation of a comprehensive analysis of complement factors, antibody screening and genetic analyses, should be part of a subsequent diagnostic work-up. Even though systematic evidence from studies is lacking, knowledge of the individual pathophysiology provides a robust foundation for the application of available therapeutic approaches for these often rapidly progressing forms of kidney disease, which frequently recur after kidney transplantation. This overview summarizes the current state of knowledge in the form of a (national German) expert consensus on the state of the art diagnostic work-up for C3 dominant glomerulonephritis and MPGN.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. C. Licht: Teilnahme an Advisory Boards, Referentenhonorare, Reisemittel und Forschungsmittel durch die Firma Alexion. B. Hohenstein: Teilnahme an Advisory Boards, Referentenhonorare und Reiseunterstützung durch die Firma Alexion. C. Hugo: Teilnahme an Advisory Boards, Referentenhonorare und Reiseunterstützung durch die Firma Alexion. M. Wiesener: Referentenhonorare und Kongressreiseunterstützung durch Alexion. C. Bergmann ist Angestellter der Firma Bioscientia. K. Amann, F. Schaefer, C. Serka, M. Kirschfink und P. Zipfel: kein Interessenkonflikt.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Bereich Nephrologie, Medizinische Klinik III, Universitätsklinikum Carl Gustav Carus, Fetscherstr. 74, 01307, Dresden, Deutschland

    B. Hohenstein & C. Hugo

  2. Abteilung für Nephrologie, The Hospital for Sick Children, Toronto, ON, Canada

    C. Licht

  3. Medizinische Klinik 4, Universitätsklinikum Erlangen, Erlangen, Deutschland

    M. Wiesener

  4. Abteilung für Nephropathologie, Pathologisches Institut, Universitätsklinikum Erlangen, Erlangen, Deutschland

    K. Amann

  5. Abteilung für Pädiatrische Nephrologie, Zentrum für Kinder- und Jugendmedizin, Universitätsklinikum Heidelberg, Heidelberg, Deutschland

    F. Schaefer

  6. Abteilung für Infektionsbiologie, Leibniz-Institut für Naturstoff-Forschung und Infektionsbiologie, Jena, Deutschland

    C. Serka & P. Zipfel

  7. Zentrum für Humangenetik, Bioscientia Institut für Medizinische Diagnostik GmbH, Ingelheim, Deutschland

    C. Bergmann

  8. Institut für Immunologie, Universitätsklinikum Heidelberg, Heidelberg, Deutschland

    M. Kirschfink

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  1. B. Hohenstein
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  2. C. Licht
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  3. M. Wiesener
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  5. F. Schaefer
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  6. C. Serka
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  7. C. Bergmann
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  8. M. Kirschfink
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  9. P. Zipfel
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  10. C. Hugo
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Correspondence to B. Hohenstein.

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Hohenstein, B., Licht, C., Wiesener, M. et al. „State-of-the-art“: C3-Glomerulopathie und membranoproliferative Glomerulonephritis. Nephrologe 10, 327–340 (2015). https://doi.org/10.1007/s11560-014-0978-6

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