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Semin Liver Dis. 2016 Aug;36(3):229-41. doi: 10.1055/s-0036-1584320. Epub 2016 Jul 28.

Histopathology of IgG4-Related Autoimmune Hepatitis and IgG4-Related Hepatopathy in IgG4-Related Disease.

Author information

1
Department of Diagnostic Pathology, Shizuoka Cancer Center, Shizuoka, Japan.
2
Department of Internal Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.
3
Department of Pathology, Kobe University Graduate School of Medicine, Kobe, Japan.
4
Department of Pathology, Kanazawa University Graduate School of Medical Science, Japan.
5
Department of Internal Medicine, Shinshu University Graduate School of Medicine, Matsumoto, Japan.

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease involving many organs; it includes IgG4-related sclerosing cholangitis and inflammatory pseudotumor in the hepatobiliary system. Two types of hepatic parenchymal involvement have been reported in IgG4-RD: IgG4-related autoimmune hepatitis (AIH) and IgG4-hepatopathy. Moreover, only three cases of IgG4-related AIH have been reported. Immunoglobulin G4-related AIH is clinicopathologically similar to AIH, except for an elevated serum IgG4 level and heavy infiltration of IgG4-positive plasma cells in the liver tissue. Interestingly, IgG4-related AIH can be complicated by well-known IgG4-RD(s). Immunoglobulin G4-hepatopathy, which includes various histopathological lesions encountered in the liver of patients with type I autoimmune pancreatitis, is classified into five histological categories: portal inflammation, large bile duct damage, portal sclerosis, lobular hepatitis, and cholestasis. Immunoglobulin G4-hepatopathy is currently a collective term covering hepatic lesions primarily or secondarily related to IgG4-related sclerosing cholangitis and type 1 autoimmune pancreatitis. In conclusion, the liver is not immune to IgG4-RD, and at least two types of hepatic involvement in IgG4-RD have been reported: IgG4-related AIH and IgG4-hepatopathy. Additional studies are required to clarify their precise clinical significance with respect to IgG4-RD and inherent liver diseases.

PMID:
27466793
DOI:
10.1055/s-0036-1584320
[Indexed for MEDLINE]

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