Open AccessImmunoglobulin G4-Hepatopathy with Acute Hepatitis-Like Onset and Marked Centrilobular Necrosis: Clinicopathologically Unique Pattern of Hepatic Injury Related to Immunoglobulin G4-Related Disease
Author(s) -
Mukai Kaori,
Nishida Tsutomu,
Adachi Shiro,
Matsumoto Kengo,
Osugi Naoto,
Sugimoto Aya,
Nakamatsu Dai,
Yamamoto Masashi,
Fukui Koji,
Tamura Hiromi,
Inada Masami
Publication year - 2021
Publication title -
case reports in gastroenterology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.247
H-Index - 18
ISSN - 1662-0631
DOI - 10.1159/000516313
Subject(s) - single case
A 69-year-old man presented with jaundice and appetite loss. Blood analyses showed elevated aminotransferase levels, hyperbilirubinemia, positivity for antinuclear antibody, elevated immunoglobulin (Ig) G4 levels, and negativity for hepatitis virus markers. Additionally, computed tomography revealed a focal enlargement of the pancreatic body and enhancement of the peripheral bile ducts. Liver biopsy showed interface hepatitis, supporting a clinical diagnosis of autoimmune hepatitis (AIH). Immunohistochemistry revealed that IgG4-bearing plasma cells accounted for more than 60% of the IgG-bearing plasma cells in the portal area. Then, we started oral prednisolone therapy. After tapering, serum transaminase levels became elevated again, and we had to adjust the dose. Azathioprine maintenance therapy was necessary to prevent relapse. We herein report a case of IgG4-hepatopathy with a clinical course similar to that of AIH with acute onset.