IgG4-related kidney disease: Clinicopathologic features, differential diagnosis, and mimics

Sarwat I. Gilani, Alessia Buglioni, Lynn D. Cornell

Research output: Contribution to journalReview articlepeer-review


IgG4-related kidney disease (IgG4-RKD) encompasses all forms of kidney disease that are part of IgG4-related disease (IgG4-RD). First recognized as IgG4-related tubulointerstitial nephritis (IgG4-TIN), and then IgG4-related membranous glomerulonephritis (IgG4-MGN), we now recognize additional patterns of interstitial nephritis, glomerular disease, and vascular disease that can be seen as part of IgG4-RKD. The clinical presentation is variable and can include acute or chronic kidney injury, proteinuria or nephrotic syndrome, mass lesion(s), and obstruction. While usually associated with other organ involvement by IgG4-RD, kidney-alone involvement is present in approximately 20 % of IgG4-RKD. Compared to IgG4-RD overall, patients with IgG4-RKD are more likely to show increased serum IgG4 or IgG, and more likely to have hypocomplementemia. In this review, we extensively cover other types of autoimmune and plasma cell-rich interstitial nephritis, mass forming inflammatory diseases of the kidney, and other mimics of IgG4-TIN, in particular ANCA-associated disease.

Original languageEnglish (US)
Pages (from-to)88-94
Number of pages7
JournalSeminars in Diagnostic Pathology
Issue number2
StatePublished - Mar 2024


  • Anti-neutrophil cytoplasmic antibody
  • Interstitial nephritis
  • Membranous glomerulonephritis
  • Membranous nephropathy
  • Renal biopsy
  • Tubulointerstitial nephritis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine


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