A case of IgG4-related autoimmune disease with multiple organ involvement

Scand J Rheumatol. 2006 Jan-Feb;35(1):69-71. doi: 10.1080/03009740500499484.

Abstract

A 52-year-old male was admitted with autoimmune pancreatitis (AIP), showing mononuclear cell infiltration in both the pancreas and salivary glands with both normal sialography and anti-SS-A/SS-B antibodies. Although the AIP improved with glucocorticoid treatment, subsequent abdominal computed tomography (CT) revealed a nodular shadow in the bilateral kidneys, which was confirmed as interstitial nephritis by renal biopsy. The patient's serum immunoglobulin G4 (IgG4) level was 10 times higher than the upper limit of the normal range. IgG4-positive mononuclear cell infiltration was detected in the salivary gland, pancreas, and kidney. A new entity proposed as 'IgG4-related autoimmune disease' was considered.

Publication types

  • Case Reports

MeSH terms

  • Autoimmune Diseases / blood
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / immunology*
  • Humans
  • Immunoglobulin G / analysis
  • Immunoglobulin G / blood*
  • Kidney / immunology
  • Male
  • Middle Aged
  • Multiple Organ Failure / blood
  • Multiple Organ Failure / immunology*
  • Pancreas / immunology
  • Pancreatitis / blood
  • Pancreatitis / immunology
  • Reference Values
  • Salivary Glands / immunology

Substances

  • Immunoglobulin G