IgG4-related kidney disease: Pathological features: Membranous glomerulonephritis

Mariam P Alexander, Lynn D. Cornell

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

IgG4-related membranous glomerulonephritis (IgG4-MGN) is the most commonly occurring glomerular disease in IgG4-related disease (IgG4-RD). Patients present with proteinuria, usually nephrotic-range, with or without renal insufficiency. The male predominance and occurrence in middle-aged to older adults parallels other recognized organ manifestations of IgG4-RD. IgG4-MGN shows histologic and immunophenotypic similarity to primary membranous glomerulonephritis, with the notable exception of negative staining for antibodies to the phospholipase A2 receptor (PLA2R). By definition, immune complex deposits containing IgG, kappa, and lambda and C3 are present along the subepithelial aspect of the glomerular basement membranes. In one series of IgG4-MGN, approximately half of the cases did not show concurrent IgG4-related tubulointerstitial nephritis (IgG4-TIN), although patients had other organ involvement by IgG4-RD. IgG4-MGN represents a pattern of tissue involvement that is distinct from most other manifestations of IgG4-RD. Although most organs affected by IgG4-RD demonstrate an “inflammatory” histologic picture, IgG4-MGN is characterized by immune complex deposits in the absence of corresponding inflammation.

Original languageEnglish (US)
Title of host publicationIgG4-Related Kidney Disease
PublisherSpringer Japan
Pages125-137
Number of pages13
ISBN (Electronic)9784431556879
ISBN (Print)9784431556862
DOIs
StatePublished - Jan 1 2016

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Keywords

  • IgG4
  • Immune complex
  • Membranous glomerulonephritis
  • Tubulointerstitial nephritis

ASJC Scopus subject areas

  • Medicine(all)
  • Immunology and Microbiology(all)

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