Immunoglobulin G4-Hepatopathy with Acute Hepatitis-Like Onset and Marked Centrilobular Necrosis: Clinicopathologically Unique Pattern of Hepatic Injury Related to Immunoglobulin G4-Related Disease
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...
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| Format: | Article |
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Karger Publishers
2021-08-01
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| Series: | Case Reports in Gastroenterology |
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| Online Access: | https://www.karger.com/Article/FullText/516313 |
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| author | Kaori Mukai Tsutomu Nishida Shiro Adachi Kengo Matsumoto Naoto Osugi Aya Sugimoto Dai Nakamatsu Masashi Yamamoto Koji Fukui Hiromi Tamura Masami Inada |
| author_facet | Kaori Mukai Tsutomu Nishida Shiro Adachi Kengo Matsumoto Naoto Osugi Aya Sugimoto Dai Nakamatsu Masashi Yamamoto Koji Fukui Hiromi Tamura Masami Inada |
| author_sort | Kaori Mukai |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-22T03:26:02Z |
| format | Article |
| id | doaj.art-bcbaaafb4d3b45ca8454656bb5acb13c |
| institution | Directory Open Access Journal |
| issn | 1662-0631 |
| language | English |
| last_indexed | 2024-12-22T03:26:02Z |
| publishDate | 2021-08-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Case Reports in Gastroenterology |
| spelling | doaj.art-bcbaaafb4d3b45ca8454656bb5acb13c2022-12-21T18:40:36ZengKarger PublishersCase Reports in Gastroenterology1662-06312021-08-0115272072810.1159/000516313516313Immunoglobulin G4-Hepatopathy with Acute Hepatitis-Like Onset and Marked Centrilobular Necrosis: Clinicopathologically Unique Pattern of Hepatic Injury Related to Immunoglobulin G4-Related DiseaseKaori Mukai0Tsutomu Nishida1https://orcid.org/0000-0003-4037-9003Shiro Adachi2Kengo Matsumoto3Naoto Osugi4Aya Sugimoto5Dai Nakamatsu6Masashi Yamamoto7Koji Fukui8Hiromi Tamura9Masami Inada10Department of Gastroenterology and Hepatology, Toyonaka Municipal Hospital, Toyonaka, JapanDepartment of Gastroenterology and Hepatology, Toyonaka Municipal Hospital, Toyonaka, JapanDepartment of Pathology, Toyonaka Municipal Hospital, Toyonaka, JapanDepartment of Gastroenterology and Hepatology, Toyonaka Municipal Hospital, Toyonaka, JapanDepartment of Gastroenterology and Hepatology, Toyonaka Municipal Hospital, Toyonaka, JapanDepartment of Gastroenterology and Hepatology, Toyonaka Municipal Hospital, Toyonaka, JapanDepartment of Gastroenterology and Hepatology, Toyonaka Municipal Hospital, Toyonaka, JapanDepartment of Gastroenterology and Hepatology, Toyonaka Municipal Hospital, Toyonaka, JapanDepartment of Gastroenterology and Hepatology, Toyonaka Municipal Hospital, Toyonaka, JapanDepartment of Pathology, Toyonaka Municipal Hospital, Toyonaka, JapanDepartment of Gastroenterology and Hepatology, Toyonaka Municipal Hospital, Toyonaka, JapanA 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.https://www.karger.com/Article/FullText/516313acute hepatitisautoimmune hepatitisimmunoglobulin g4-hepatopathyazathioprine |
| spellingShingle | Kaori Mukai Tsutomu Nishida Shiro Adachi Kengo Matsumoto Naoto Osugi Aya Sugimoto Dai Nakamatsu Masashi Yamamoto Koji Fukui Hiromi Tamura Masami Inada Immunoglobulin G4-Hepatopathy with Acute Hepatitis-Like Onset and Marked Centrilobular Necrosis: Clinicopathologically Unique Pattern of Hepatic Injury Related to Immunoglobulin G4-Related Disease Case Reports in Gastroenterology acute hepatitis autoimmune hepatitis immunoglobulin g4-hepatopathy azathioprine |
| title | Immunoglobulin G4-Hepatopathy with Acute Hepatitis-Like Onset and Marked Centrilobular Necrosis: Clinicopathologically Unique Pattern of Hepatic Injury Related to Immunoglobulin G4-Related Disease |
| title_full | Immunoglobulin G4-Hepatopathy with Acute Hepatitis-Like Onset and Marked Centrilobular Necrosis: Clinicopathologically Unique Pattern of Hepatic Injury Related to Immunoglobulin G4-Related Disease |
| title_fullStr | Immunoglobulin G4-Hepatopathy with Acute Hepatitis-Like Onset and Marked Centrilobular Necrosis: Clinicopathologically Unique Pattern of Hepatic Injury Related to Immunoglobulin G4-Related Disease |
| title_full_unstemmed | Immunoglobulin G4-Hepatopathy with Acute Hepatitis-Like Onset and Marked Centrilobular Necrosis: Clinicopathologically Unique Pattern of Hepatic Injury Related to Immunoglobulin G4-Related Disease |
| title_short | Immunoglobulin G4-Hepatopathy with Acute Hepatitis-Like Onset and Marked Centrilobular Necrosis: Clinicopathologically Unique Pattern of Hepatic Injury Related to Immunoglobulin G4-Related Disease |
| title_sort | immunoglobulin g4 hepatopathy with acute hepatitis like onset and marked centrilobular necrosis clinicopathologically unique pattern of hepatic injury related to immunoglobulin g4 related disease |
| topic | acute hepatitis autoimmune hepatitis immunoglobulin g4-hepatopathy azathioprine |
| url | https://www.karger.com/Article/FullText/516313 |
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