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Pathophysiology

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IgG4-Related Sclerosing Cholangitis

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Abstract

During the discovery process of IgG4-related disease (IgG4-RD), the bile duct appeared to be one of the target organs commonly affected by this condition [1–3]. Sclerosing cholangitis is a central biliary manifestation of IgG4-RD [1–3]. Although clinical manifestations of IgG4-RD vary widely among patients, underlying immune reactions and pathophysiology are supposed to be similar in any organs given the almost identical histopathological changes. Similar to other immune-mediated conditions, a likely pathogenetic mechanism is that the disease develops in genetically susceptible individuals exposed to external or endogenous antigens [4]. In this review, our current understanding of the molecular features of this emerging biliary disease is summarized. Data obtained from not only IgG4-related sclerosing cholangitis (IgG4-SC) but also IgG4-RD at other anatomical sites are discussed [4, 5].

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Abbreviations

IgG4-AIP:

IgG4-related autoimmune pancreatitis

IgG4-RD:

IgG4-related disease

IgG4-SC:

IgG4-related sclerosing cholangitis

PSC:

Primary sclerosing cholangitis

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None to declare.

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Authors and Affiliations

  1. Department of Diagnostic Pathology, Kobe University Graduate School of Medicine, Kobe, Japan

    Yoh Zen

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  1. Yoh Zen
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Correspondence to Yoh Zen .

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Editors and Affiliations

  1. Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Bunkyo-ku, Tokyo, Japan

    Terumi Kamisawa

  2. Department of Internal Medicine, ASAN Medical Center, Seoul, Korea (Republic of)

    Myung-Hwan Kim

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Zen, Y. (2019). Pathophysiology. In: Kamisawa, T., Kim, MH. (eds) IgG4-Related Sclerosing Cholangitis. Springer, Singapore. https://doi.org/10.1007/978-981-10-4548-6_3

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  • DOI: https://doi.org/10.1007/978-981-10-4548-6_3

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  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-10-4547-9

  • Online ISBN: 978-981-10-4548-6

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