Semin Liver Dis 2016; 36(03): 229-241
DOI: 10.1055/s-0036-1584320
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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

Yasuni Nakanuma
1  Department of Diagnostic Pathology, Shizuoka Cancer Center, Shizuoka, Japan
Yoji Ishizu
2  Department of Internal Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan
Yoh Zen
3  Department of Pathology, Kobe University Graduate School of Medicine, Kobe, Japan
Kenichi Harada
4  Department of Pathology, Kanazawa University Graduate School of Medical Science, Japan
Takeji Umemura
5  Department of Internal Medicine, Shinshu University Graduate School of Medicine, Matsumoto, Japan
› Author Affiliations
Further Information

Publication History

Publication Date:
28 July 2016 (online)


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.