{"title":"IgG4-AIH with multiple inflammatory pseudotumors and decreased OATP1B3 expression.","authors":"Ryusuke Hanafusa, Kazuto Tajiri, Nozomu Muraishi, Kenichi Hirabayashi, Koichi Tsuneyama, Ichiro Yasuda","doi":"10.1007/s12328-025-02161-8","DOIUrl":null,"url":null,"abstract":"<p><p>Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease caused by an autoimmune response to hepatocytes, predominantly affecting middle-aged and older women. A subset of AIH cases has been associated with immunoglobulin G4-related disease (IgG4-RD), referred to as IgG4-AIH. This rare condition is characterized by IgG4-positive plasma cell infiltration in the portal area and elevated serum IgG4 levels. Despite its rarity, IgG4-AIH has been proposed as a distinct disease entity. Here, we report a case of IgG4-AIH associated with multiple inflammatory pseudotumors (IPTs) and decreased organic anion transporting peptide (OATP) 1B3 expression. Hepatic IPTs are typically composed of lymphoplasmacytic and fibroblastic accumulations. In the present case, OATP1B3 expression was relatively decreased without such accumulation according to IgG4-related inflammation, suggesting that IgG4-related inflammation may regulate OATP1B3 expression.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":"760-766"},"PeriodicalIF":0.9000,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical Journal of Gastroenterology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1007/s12328-025-02161-8","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/6/17 0:00:00","PubModel":"Epub","JCR":"Q4","JCRName":"GASTROENTEROLOGY & HEPATOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease caused by an autoimmune response to hepatocytes, predominantly affecting middle-aged and older women. A subset of AIH cases has been associated with immunoglobulin G4-related disease (IgG4-RD), referred to as IgG4-AIH. This rare condition is characterized by IgG4-positive plasma cell infiltration in the portal area and elevated serum IgG4 levels. Despite its rarity, IgG4-AIH has been proposed as a distinct disease entity. Here, we report a case of IgG4-AIH associated with multiple inflammatory pseudotumors (IPTs) and decreased organic anion transporting peptide (OATP) 1B3 expression. Hepatic IPTs are typically composed of lymphoplasmacytic and fibroblastic accumulations. In the present case, OATP1B3 expression was relatively decreased without such accumulation according to IgG4-related inflammation, suggesting that IgG4-related inflammation may regulate OATP1B3 expression.
期刊介绍：
The journal publishes Case Reports and Clinical Reviews on all aspects of the digestive tract, liver, biliary tract, and pancreas. Critical Case Reports that show originality or have educational implications for diagnosis and treatment are especially encouraged for submission. Personal reviews of clinical gastroenterology are also welcomed. The journal aims for quick publication of such critical Case Reports and Clinical Reviews.
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