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20.06.2023 | IgG4-assoziierte Erkrankungen | CME

IgG4-assoziierte Nierenerkrankungen

verfasst von: Christina Thompson, Frank O. Henes, Oliver M. Steinmetz, Dr. med. Simon Melderis

Erschienen in: Die Nephrologie | Ausgabe 4/2023

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Zusammenfassung

Die Immunglobulin(Ig)-G4-assoziierte Erkrankung (IgG4-RD) ist eine entzündliche Systemerkrankung, die durch eine chronische Aktivierung des Immunsystems mit konsekutiver Gewebefibrose gekennzeichnet ist und eine Vielzahl verschiedener Organsysteme betreffen kann. Die häufigste Form der Nierenbeteiligung (IgG4-RKD) ist die tubulointerstitielle Nephritis (IN) mit charakteristischen Infiltraten von IgG4-positiven Plasmazellen im Interstitium und storiformer Fibrose. Seltener finden sich Formen mit glomerulärer Beteiligung sowie Harnstauungsnieren durch retroperitoneale Fibrose. Klinisch präsentiert sich die IgG4-RKD meist unspezifisch, und es findet sich lediglich eine eingeschränkte Nierenfunktion bei unauffälliger Urinanalyse. Ein erhöhtes Serum-IgG4 kann die Verdachtsdiagnose erhärten. Mittels Schnittbildgebung lassen sich zudem häufig charakteristische kortexnahe Läsionen nachweisen, welche schwer von Malignomen abzugrenzen sind. Obwohl man typischerweise ein schnelles klinisches Ansprechen der IgG4-RKD auf eine Therapie mit Glukokortikoiden findet, kommt es oft nur zu einer partiellen Verbesserung der Nierenfunktion. Rezidive sind häufig, und das optimale Management der Nachsorge und der Langzeittherapie ist unklar. Im Rahmen dieses Beitrags sollen die verschiedenen Formen der IgG4-RKD näher erläutert werden.

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Titel: IgG4-assoziierte Nierenerkrankungen
verfasst von: Christina Thompson
Frank O. Henes
Oliver M. Steinmetz
Dr. med. Simon Melderis
Publikationsdatum: 20.06.2023
Verlag: Springer Medizin
Schlagwörter: IgG4-assoziierte Erkrankungen
Interstitielle Nephritis
Retroperitoneale Fibrose
IgG4-assoziierte Pankreatitis
Glucocorticoide
Ultraschall
Magnetresonanztomografie
Computertomografie
Retroperitoneale Fibrose
Glomerulonephritiden
DMARDs
Rituximab
Nephrologische Erkrankungen in der Hausarztpraxis
Erschienen in: Die Nephrologie / Ausgabe 4/2023
Print ISSN: 2731-7463
Elektronische ISSN: 2731-7471
DOI: https://doi.org/10.1007/s11560-023-00664-4

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