Die membranöse Glomerulonephritis ist eine histologisch, pathophysiologisch und klinisch gut abgrenzbare Erkrankung. Histologisch finden sich Immunglobulin- (IgG4) und C3-Ablagerungen vornehmlich subendothelial, die die glomerulären Basalmembran diffus verdickt erscheinen lässt. In der Elektronenmikroskopie erweisen sich die subendothelialen Ablagerungen als elektronendicht. Pathophysiologisch scheinen zumindest bei einem Teil der Patienten Autoantikörper gegen Podozytenantigene insbesondere das PLA2R1 eine Rolle zu spielen. Die Komplementaktievierung erfolgt wahrscheinlich über den Lectin-Weg. Klinisch zeigen die Patienten meist ein ausgeprägtes nephrotisches Syndrom.
Es werden zwei Formen unterschieden die primäre oder idiopathische Form (75%) und die sekundäre form infolge anderer Erkrankungen: systemischer Lupus erythematodes, Hepatitis B, Medikamente oder bösartige Tumoren.
Autoantikörper gegen podozytäre Antigene spielen eine wichtige pathogenetische Rolle. Ihre Entstehung korreliert sehr stark mit Polymorphismen in den Genen PLA2R1 und HLA-DQA1.
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