IgG4-related tubulointerstitial nephritis and hepatic inflammatory pseudotumor without hypocomplementemia

Intern Med. 2011;50(11):1239-44. doi: 10.2169/internalmedicine.50.5102. Epub 2011 Jun 1.

Abstract

Immunoglobulin G4 (IgG4)-related tubulointerstitial nephritis (TIN) is often accompanied by autoimmune pancreatitis (AIP) or chronic sclerosing dacryoadenitis and sialoadenitis. However, IgG4-related TIN without AIP or lacrimal and/or salivary gland lesions has not been well recognized. Here, we report a case of IgG4-related TIN associated with hepatic inflammatory pseudotumor without AIP or lacrimal and/or salivary gland lesions. A 58-year-old Japanese man with epigastralgia underwent contrast-enhanced computed tomography (CT), which revealed multiple low-density lesions in both kidneys and a low density hepatic mass. Laboratory tests showed an extremely high level of serum IgG4. Percutaneous renal and hepatic biopsies showed diffuse infiltration of lymphocytes and IgG4-positive plasma cells with fibrosis in both tissues. Two months after administration of oral prednisolone, both lesions decreased in size on follow-up CT, and the serum creatinine level also improved. No recurrence has been detected for two years with a maintenance dose of prednisolone.

Publication types

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

MeSH terms

  • Biopsy
  • Comorbidity
  • Glomerulonephritis, Membranoproliferative*
  • Glucocorticoids / therapeutic use
  • Granuloma, Plasma Cell / diagnosis
  • Granuloma, Plasma Cell / drug therapy
  • Granuloma, Plasma Cell / epidemiology*
  • Humans
  • Immunoglobulin G / blood*
  • Kidney / pathology
  • Liver / pathology
  • Liver Diseases / diagnosis
  • Liver Diseases / drug therapy
  • Liver Diseases / epidemiology*
  • Male
  • Middle Aged
  • Nephritis, Interstitial / drug therapy
  • Nephritis, Interstitial / epidemiology*
  • Nephritis, Interstitial / immunology*
  • Prednisolone / therapeutic use
  • Tomography, X-Ray Computed
  • Treatment Outcome

Substances

  • Glucocorticoids
  • Immunoglobulin G
  • Prednisolone