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].


Autoimmune pancreatitis IgG4-related disease T cell B cell Pathogenesis 



IgG4-related autoimmune pancreatitis


IgG4-related disease


IgG4-related sclerosing cholangitis


Primary sclerosing cholangitis


Conflict of Interest

None to declare.


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Copyright information

© Springer Science+Business Media Singapore 2019

Authors and Affiliations

  1. 1.Department of Diagnostic PathologyKobe University Graduate School of MedicineKobeJapan

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