Update membranöse Glomerulonephritis

Was ist neu?

Diagnose von PLA₂R1- und THSD7A-Antikörper-positiver membranöser Glomerulonephritis Der Nachweis von PLA₂R1- und THSD7A-Antikörpern im Blut sowie die immunhistologische Analyse von Nierenbiopsien für die entsprechenden Antigene ermöglichen in praktisch 100 % der Fälle die korrekte Diagnose einer PLA₂R1- oder THSD7A-assoziierten membranösen Glomerulonephritis (MGN) [1] [2] [3]. Die Entscheidung für oder gegen eine Nierenbiopsie kann individualisiert getroffen werden, unter Berücksichtigung der Vorerkrankungen, Prozedurrisiken, klinischen und laborchemischen Befunden usw. [3]. Die pathogenetische oder diagnostische Rolle weiterer Antigene wird weiterhin erforscht. Hierbei konnte kürzlich auch erstmals ein PLA₂R1-Mausmodell etabliert werden, welches nun die experimentellen Möglichkeiten erweitert [4] [5] [6] [7].

Klinische Rolle der PLA₂R1-Antikörper PLA₂R1-Antikörperspiegel sind Prädiktoren für eine Remission der Proteinurie sowie die Entwicklung einer Niereninsuffizienz, Dialysepflichtigkeit und eines Relapses der Erkrankung [8]. Die Behandlungsstrategie der MGN basiert zunehmend auf der Höhe der PLA₂R1-Antikörperspiegel [9].

Therapie der membranösen Glomerulonephritis Rituximab ist nicht unterlegen für die Induktion einer Proteinurieremission nach 12 Monaten und überlegen für den Erhalt der Proteinurieremission nach 24 Monaten verglichen mit Ciclosporin A [10]. Die Entwicklung neuer Therapiestrategien, die auf die Krankheitspathogenese und -aktivität des einzelnen Patienten gerichtet sind, bleibt bei der MGN hoch relevant.

Abstract

Membranous nephropathy is an autoimmune disease caused in most cases by binding of circulating antibodies to antigens on podocytes. PLA₂R1 and THSD7A have been identified as target antigens in 70–80 % and 2–3 % of patients, respectively. The detection of PLA₂R1- and THSD7A-antibodies in the blood and staining of renal biopsies for the respective antigens allow the correct diagnosis of PLA₂R1- and THSD7A-associated membranous nephropathy, respectively, in practically 100 % of cases. Measurement of PLA₂R1- and THSD7A-antibodies is helpful in order to further individualize the decision whether to perform a renal biopsy in patients with suspected membranous nephropathy. PLA₂R1-antibody level is a strong predictor for remission of proteinuria, loss of renal function and relapse of disease. Moreover, treatment decisions in patients with PLA₂R1-associated MN are increasingly based on the PLA₂R1-antibody levels. Rituximab was shown to be non-inferior to ciclosporine A to induce remission of proteinuria after 12 months. After 24 months rituximab was superior to ciclosporine A for the same endpoint. Development of novel treatment strategies, focusing on disease pathogenesis, remains highly relevant for these patients.

Schlüsselwörter

membranöse Glomerulonephritis - PLA²R1-Antikörper - THSD7A-Antikörper - Immunsuppression

Key words

membranous nephropathy - PLA₂R1-antibody - THSD7A-antibody - immunosuppression

• Literatur

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