Renal involvement by IgG4-related disease

Lynn D. Cornell

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

IgG4-related disease (IgG4-RD) is a recently recognized systemic immune-mediated disease, typically characterized by mass-forming fibroinflammatory lesions that may affect nearly any organ. In the kidney, IgG4-RD is most often manifested as IgG4-related tubulointerstitial nephritis (IgG4-TIN), which can present as mass lesions, renal insufficiency, or both. IgG4-TIN has a histologic appearance to other organs involved by IgG4-RD and shows a plasma cell-rich tubulointerstitial nephritis with storiform fibrosis; most cases also show tubular basement membrane immune complex deposits, which is a helpful feature on biopsy. IgG4-TIN, like fibroinflammatory manifestations of IgG4-RD in other organs, typically shows a brisk response to steroid therapy, although there is a high relapse rate. Glomerular involvement by IgG4-RD, especially membranous glomerulonephritis, has been recognized more recently.

Original languageEnglish (US)
Title of host publicationCore Concepts in Parenchymal Kidney Disease
PublisherSpringer New York
Pages263-268
Number of pages6
Volume9781461481669
ISBN (Print)9781461481669, 1461481651, 9781461481652
DOIs
StatePublished - Jul 1 2014

ASJC Scopus subject areas

  • Medicine(all)

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    Cornell, L. D. (2014). Renal involvement by IgG4-related disease. In Core Concepts in Parenchymal Kidney Disease (Vol. 9781461481669, pp. 263-268). Springer New York. https://doi.org/10.1007/978-1-4614-8166-9_17