title: Histopathology of IgG4-Related Autoimmune Hepatitis and IgG4-Related Hepatopathy in IgG4-Related Disease.
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.
authors.affiliation: Department of Diagnostic Pathology, Shizuoka Cancer Center, Shizuoka, Japan.
authors.forename: Yasuni
authors.initials: Y
authors.lastname: Nakanuma
authors.affiliation: Department of Internal Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Yoji
authors.initials: Y
authors.lastname: Ishizu
authors.affiliation: Department of Pathology, Kobe University Graduate School of Medicine, Kobe, Japan.
authors.forename: Yoh
authors.initials: Y
authors.lastname: Zen
authors.affiliation: Department of Pathology, Kanazawa University Graduate School of Medical Science, Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Harada
authors.affiliation: Department of Internal Medicine, Shinshu University Graduate School of Medicine, Matsumoto, Japan.
authors.forename: Takeji
authors.initials: T
authors.lastname: Umemura
mesh_terms: D000328:Adult; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001649:Bile Duct Diseases / Q000276:immunology / Q000473:pathology; D015209:Cholangitis, Sclerosing / Q000150:complications; D005260:Female; D019693:Hepatitis, Autoimmune / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood* / Q000276:immunology; D008107:Liver Diseases / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D012008:Recurrence; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography
chemical_list: D000893:Anti-Inflammatory Agents; D007074:Immunoglobulin G; D011241:Prednisone