Autoimmune hepatitis and IgG4-related disease.

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.

• Authors: Kosuke Minaga, Tomohiro Watanabe, Hobyung Chung, Masatoshi Kudo
• Journal: World journal of gastroenterology (World J Gastroenterol) Vol. 25 Issue 19 Pg. 2308-2314 (May 21 2019) ISSN: 2219-2840 [Electronic] United States
• PMID: 31148902 (Publication Type: Journal Article, Review)

Chemical References

• Immunoglobulin G

Topics

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