IgG4-related renal disease: Clinical and pathological characteristics

Naoto Kuroda, Tomoya Nao, Hideo Fukuhara, Takashi Karashima, Keiji Inoue, Yoshinori Taniguchi, Mai Takeuchi, Yoh Zen, Yasuharu Sato, Kenji Notohara, Tadashi Yoshino

Research output: Contribution to journalReview articlepeer-review

20 Citations (Scopus)


IgG4-related disease is a recently established systemic condition. Tubulointerstitial nephritis is the most common renal manifestation. Glomerular lesions, particularly membranous glomerulonephritis, can develop simultaneously. Some patients present with serological renal dysfunction associated with elevated IgG or IgE levels and hypocomplementemia, while others are incidentally found to have abnormalities in kidneys on imaging. A majority of patients with IgG4-related kidney disease have similar lesions at other anatomical sites, which help us to suspect this condition. Serum IgG4 elevation (>135 mg/dL) is the most, although not entirely, specific marker for the diagnosis. Imaging findings varies from small nodules to bilateral diffuse abnormalities. In addition to the renal parenchyma, the renal pelvis and perirenal adipose tissue can be affected. Histological features include dense lymphoplasmacytic infiltration, storiform or "bird's eye" fibrosis (highlighted by PAM stain), and IgG4-positive plasma cell infiltration (>10 cells/high-power field and IgG4/IgG-positive cell ratio >40%). Immune complex deposition is detectable in the tubular basement membrane by immunofluorescence and/or electron microscopy. Patients usually respond well to corticosteroids, but highly active diseases may require other immunosuppressive therapies. Further investigations will be required to fully understand pathophysiology underlying this emerging condition.

Original languageEnglish
Pages (from-to)6379-6385
Number of pages7
JournalInternational Journal of Clinical and Experimental Pathology
Issue number9
Publication statusPublished - 2014


  • IgG4-related disease
  • Kidney
  • Review

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology


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