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• W4385981630 abstract "IgG4-related cholangitis (IRC) is the major hepatobiliary manifestation of IgG4-related disease (IgG4-RD), a systemic fibroinflammatory disorder. The pathogenesis of IgG4-RD and IRC is currently viewed as multifactorial with evidence of a genetic predisposition and also environmental factors such as blue-collar work being a major risk factor. Various autoantigens have been described in IgG4-RD including annexin A11 and laminin 511-E8, which may partially exert a protective function in cholangiocytes through enhancing secretion and barrier function, respectively. For the other recently described autoantigens, galectin-3 and prohibitin 1, a distinct role in cholangiocytes appears less apparent. In relation to these autoantigens, oligoclonal expansion of IgG4+ plasmablasts are present in IRC patients and disappear upon successful treatment. More recently, specific T cell subtypes including regulatory T cells, follicular T helper 2 cells, peripheral T helper cells and cytotoxic CD8+ and CD4+ SLAMF7+ T cells have been implicated in the pathogenesis of IgG4-RD. The clinical presentation of IRC often mimics other biliary diseases such as primary sclerosing cholangitis or cholangiocarcinoma, which may lead to inappropriate medical and potentially invalidating surgical interventions. As specific biomarkers are lacking, diagnosis is made according to the HISORt criteria comprising histopathology, imaging, serology, other organ manifestations and response to therapy. Treatment of IRC aims to prevent or alleviate organ damage and to improve symptoms and consists of (i) remission induction, (ii) remission maintenance and (iii) long-term management. For remission induction glucocorticosteroids are highly effective, after which immunomodulators can be introduced for maintenance of remission as glucocorticosteroid sparing alternatives. Increased insight into the pathogenesis of IRC will lead to improved diagnosis and novel therapeutic strategies in the future." @default.
• W4385981630 created "2023-08-19" @default.
• W4385981630 creator A5018461629 @default.
• W4385981630 creator A5038191402 @default.
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• W4385981630 creator A5084875431 @default.
• W4385981630 date "2023-08-01" @default.
• W4385981630 modified "2023-10-14" @default.
• W4385981630 title "IgG4-related cholangitis - a mimicker of fibrosing and malignant cholangiopathies" @default.
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