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Journal of Nephrology

Erschienen in:

01.08.2016 | Review

IgG4-related nephropathy

verfasst von: Giacomo Quattrocchio, Dario Roccatello

Erschienen in: Journal of Nephrology | Ausgabe 4/2016

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Abstract

IgG4-related disease (IgG4-RD) is a recently recognized disorder, often with multiple organ involvement, characterized by dense tissue infiltration of IgG4-positive plasma cells, storiform fibrosis, obliterative phlebitis and frequently elevated serum IgG4 concentration. The kidney can be involved either directly or indirectly. The most frequent direct renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis (TIN) and membranous glomerulonephropathy. Retroperitoneal fibrosis (RPF) is another condition that is frequently IgG4-related and that can indirectly affect the kidney causing ureteral obstruction and hydronephrosis. Contrast-enhanced computerized tomography, magnetic resonance imaging and ¹⁸F-fluorodeoxyglucose positron emission tomography/computed tomography show different imaging findings and are useful tools for monitoring therapeutic response. Steroid treatment is the first line of therapy, but relapsing or refractory forms of the disease are frequently observed and require more aggressive therapeutic approaches. At our centre, we treated three cases of aggressive IgG4-related TIN and two cases of IgG4-related RPF with an intensified, immune suppressive protocol, obtaining good results without severe adverse effects.

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Titel: IgG4-related nephropathy
verfasst von: Giacomo Quattrocchio
Dario Roccatello
Publikationsdatum: 01.08.2016
Verlag: Springer International Publishing
Erschienen in: Journal of Nephrology / Ausgabe 4/2016
Print ISSN: 1121-8428
Elektronische ISSN: 1724-6059
DOI: https://doi.org/10.1007/s40620-016-0279-4

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