[Coexistence of IgG4-related autoimmune hepatitis and inflammatory pseudotumors of the liver:a case report]

Nihon Shokakibyo Gakkai Zasshi. 2020;117(6):532-541. doi: 10.11405/nisshoshi.117.532.
[Article in Japanese]

Abstract

IgG4-related autoimmune hepatitis (IgG4-AIH) is characterized by hepatic inflammation and is considered an IgG4-related disease. Several inflammatory pseudotumors (IPTs) are also considered as IgG4-related diseases;however, there have been no reports of cases wherein both diseases occurred concurrently. An older adult with liver dysfunction was admitted to the hospital and was diagnosed with IgG4-AIH following a liver biopsy;IgG4-positive plasma cell infiltration in the portal tract and high serum IgG4 concentration were detected. A few months following biopsy, imaging studies revealed two IPTs in the liver. The patient was diagnosed with cryptogenic organized pneumonia several months after imaging and was treated with steroids in a different hospital. Her liver dysfunction improved, and one of the two IPTs disappeared in response to steroid treatment. The following is an account of a rare case of IgG4-AIH with IPTs of the liver.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Autoimmune Diseases*
  • Female
  • Granuloma, Plasma Cell*
  • Hepatitis, Autoimmune*
  • Humans
  • Immunoglobulin G
  • Liver Diseases*

Substances

  • Immunoglobulin G