Renal involvement by IgG4-related disease

Lynn D. Cornell

Research output: Chapter in Book/Report/Conference proceedingChapter


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
Number of pages6
ISBN (Print)9781461481669, 1461481651, 9781461481652
StatePublished - Jul 1 2014

ASJC Scopus subject areas

  • Medicine(all)


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