Nephropathy in IgG4-related systemic disease

Simon J W Watson, David A S Jenkins, Christopher O S Bellamy

Research output: Contribution to journalArticlepeer-review

Abstract / Description of output

Lymphoplasmacytic sclerosing pancreatitis ("autoimmune" pancreatitis) is the best-known manifestation of an unusual corticosteroid-sensitive systemic fibrosclerotic disease that is associated with high plasma immunoglobulin G4 (IgG4) and tissue infiltration with IgG4-secreting plasma cells. Pancreatic and biliary manifestations of this condition are well-reported, but reports of other systemic involvement are few. We report here a case of initially unrecognized autoimmune pancreatitis followed 5 years later by a focal sclerosing lymphoplasmacytic tubulointerstitial nephritis and concurrent membranous nephropathy. The patient presented with hypertension, a raised serum creatinine, proteinuria, elevated serum IgG4, and eosinophilia. Immunolabeling of renal tissue showed numerous IgG4 positive plasma cells with peritubular and glomerular subepithelial IgG4 deposition. On steroid therapy serum IgG4 levels normalized, the eosinophilia resolved, and there was improvement in symptomatic wheeze, dry eyes, serum creatinine, and liver function tests. This case highlights a distinctive and potentially treatable form of interstitial nephritis manifesting from a systemic immune disorder, and provides circumstantial evidence to support the notion that dysregulated IgG4 can precipitate the development of a form of membranous nephropathy.
Original languageEnglish
Pages (from-to)1472-7
Number of pages6
JournalThe American Journal of Surgical Pathology
Issue number11
Publication statusPublished - Nov 2006

Keywords / Materials (for Non-textual outputs)

  • Adrenal Cortex Hormones
  • Aged
  • Autoimmune Diseases
  • Diabetes Mellitus, Type 1
  • Glomerulonephritis, Membranous
  • Humans
  • Hypertension
  • Immunoglobulin G
  • Kidney
  • Male
  • Nephritis
  • Pancreatitis
  • Proteinuria


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