C3-Glomerulopathie und MPGN – aktuelle Klassifikationen

Abstract

Membranoproliferative glomerulonephritis (MPGN) and glomerulopathy with dominant C3 deposits are very rare autoimmune disorders of the kidney that had been classified in its current form in 2010 due to a better understanding of the underlying pathophysiology.

Today, the immune complex-associated membranoproliferative glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G) represent a disease spectrum which is heterogeneous in terms of pathophysiology and the clinical time course. Interestingly, recent research demonstrated more common pathophysiological aspects with respect to secondary causes, autoantibodies, and genetics of IC-MPGN and C3G than it had been suggested with the creation of the 2010 classification.

This knowledge of the underlying pathophysiology is of specific importance for the identification of potential secondary causes. Therefore, following histologic diagnosis a comprehensive complement analysis, accompanied by antibody screening and human genetics should be carried out consistently. The published evidence provides a robust basis for the use of available therapeutic approaches for these often rapidly progressive and relapsing diseases.

Sehr selten wird bei Patienten die Diagnose einer C3-Glomerulopathie oder membranoproliferativen Glomerulonephritis gestellt. Durch die Relevanz des Komplementsystems für diese Erkrankungen ist die Kenntnis der Pathophysiologie von großer Bedeutung. Dieser Beitrag trägt aktuelle Entwicklungen zum Verständnis dieser Krankheitsbilder zusammen und soll Hilfestellung bei der Aufarbeitung entsprechender Fälle geben.

Schlüsselwörter

C3-Glomerulopathie - membranoproliferative Glomerulonephritis - Komplement - Genetik - Diagnostik - Dense Deposit Disease - C3-Glomerulonephritis - CFHR

Keywords

membranoproliferative glomerulonephritis - MPGN - C3 glomerulopathy - C3G - immune complex-associated membranoproliferative glomerulonephritis IC-MPGN

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