Autoimmune hepatitis and IgG4-related disease

World J Gastroenterol. 2019 May 21;25(19):2308-2314. doi: 10.3748/wjg.v25.i19.2308.

Abstract

IgG4-related disease (IgG4-RD) is a chronic-fibroinflammatory disorder affecting a wide range of organs. Elevation of serum IgG4 concentrations and abundant infiltration of IgG4-expressing plasma cells are key diagnostic features of this autoimmune disease. Although common organ involvement of IgG4-RD includes the salivary glands, pancreas, and bile duct, hepatic involvement is less well established. Recently, five studies identified a subtype of autoimmune hepatitis (AIH), called IgG4-associated AIH (IgG4-AIH). IgG4-AIH is diagnosed based on significant accumulation of IgG4-expressing plasmacytes in the liver in patients who met the diagnostic criteria for classical AIH. Although four of the five reports regarded IgG4-AIH based on hepatic accumulation of IgG4-positive cells alone, one report diagnosed IgG4-AIH based on both hepatic accumulation of IgG4-positive cells and elevated serum concentrations of IgG4. IgG4-AIH diagnosed based on the latter criteria may be a hepatic manifestation of IgG4-RD whereas IgG4-AIH diagnosed based on the former criteria may be a subtype of AIH. In this review article, we summarize and discuss clinicopathological features of IgG4-AIH.

Keywords: Autoimmune hepatitis; IgG4; IgG4-associated autoimmune hepatitis; IgG4-related disease.

Publication types

  • Review

MeSH terms

  • Hepatitis, Autoimmune / blood
  • Hepatitis, Autoimmune / diagnosis
  • Hepatitis, Autoimmune / immunology*
  • Humans
  • Immunoglobulin G / blood*
  • Immunoglobulin G / immunology
  • Immunoglobulin G / metabolism
  • Immunoglobulin G4-Related Disease / blood
  • Immunoglobulin G4-Related Disease / complications*
  • Immunoglobulin G4-Related Disease / immunology
  • Liver / cytology
  • Liver / immunology
  • Liver / pathology
  • Plasma Cells / immunology
  • Plasma Cells / metabolism

Substances

  • Immunoglobulin G