IgG4-related kidney disease

Lynn D. Cornell

Research output: Contribution to journalReview articlepeer-review


IgG4-related disease (IgG4-RD) is a recently recognized systemic immune-mediated disease that can affect nearly any organ or tissue. The most common manifestation in the kidney is IgG4-related tubulointerstitial nephritis (IgG4-TIN), which can present as renal insufficiency, renal mass lesions, or both. Histologically, IgG4-TIN is a plasma cell-rich interstitial inflammatory infiltrate with mononuclear cells, eosinophils, and increased IgG4+ plasma cells, along with expansile interstitial fibrosis that often has a " storiform" appearance. Tubular basement membrane immune complex deposits, best visualized on immunofluorescence staining, are present in most cases. IgG4-TIN usually shows a rapid response to steroid therapy. Glomeruli may be affected by IgG4-RD, usually in the form of membranous glomerulonephritis; other glomerular lesions have also been described. This review describes the different histopathologic patterns of renal involvement by IgG4-RD, with associated clinical, radiographic, and serologic features.

Original languageEnglish (US)
Pages (from-to)166-174
Number of pages9
JournalDiagnostic Histopathology
Issue number5
StatePublished - May 1 2013


  • Autoimmune pancreatitis
  • IgG4-related sclerosing disease
  • Immune complex
  • Interstitial nephritis
  • Membranous glomerulonephritis
  • Membranous nephropathy

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology


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