IgG4-Related Kidney Disease and IgG4-Related Retroperitoneal Fibrosis

Semin Liver Dis. 2016 Aug;36(3):283-90. doi: 10.1055/s-0036-1584316. Epub 2016 Jul 28.


Immunoglobulin G4-related kidney disease (IgG4-RKD) is the collective name encompassing renal parenchymal and renal pelvic lesions. The hallmark of renal parenchymal lesions of IgG4-related disease is plasma cell-rich tubulointerstitial nephritis with numerous IgG4-positive plasma cells and characteristic fibrosis. In addition, glomerular lesions are sometimes present, with membranous glomerulonephritis being the most common. Although IgG4-RKD shows good responsiveness to corticosteroid therapy, follow-up imaging studies have revealed that partial cortical scars persist when the start of therapy is delayed. In this review, the authors provide an overview of the latest knowledge of IgG4-RKD, focusing in particular on its pathological and imaging characteristic features.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Female
  • Humans
  • Immunoglobulin G / blood*
  • Kidney / diagnostic imaging
  • Kidney / pathology*
  • Male
  • Nephritis, Interstitial / diagnosis
  • Nephritis, Interstitial / pathology*
  • Positron-Emission Tomography
  • Retroperitoneal Fibrosis / diagnostic imaging
  • Retroperitoneal Fibrosis / pathology*
  • Retroperitoneal Fibrosis / therapy
  • Tomography, X-Ray Computed


  • Adrenal Cortex Hormones
  • Immunoglobulin G