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Immunopathology of Bile Duct Lesions of Primary Biliary Cirrhosis

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Pathology of the Bile Duct

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Abstract

Primary biliary cirrhosis (PBC) is an organ-specific autoimmune disease that predominantly affects women and is characterized by chronic progressive destruction of the small intrahepatic bile ducts with portal inflammation and ultimately fibrosis. The serologic hallmark of PBC is the presence of anti-mitochondrial autoantibodies (AMA). Several mechanisms may now be proposed regarding the immune-mediated bile duct damage in PBC, including the possible roles of T cells, B cells, AMA, and other cell phenotypes. Weakness of biliary epithelial cells in association with apoptosis, senescence, and autophagy has also been noted recently. In PBC, several complex steps and mechanisms may be involved in the induction and progression of cholangitis and biliary degeneration, followed by bile duct loss.

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Authors and Affiliations

  1. Department of Pathology and Laboratory Medicine, Institute of Biomedical Sciences, Tokushima University Graduate School, 3-18-15 Kuramoto, Tokushima-shi, Tokushima, 770-8503, Japan

    Hayato Baba, Ayumi Sugitani, Ryusei Takahashi, Kouki Kai & Koichi Tsuneyama

  2. Department of General Medical Practice and Laboratory Diagnostic Medicine, Akita University Graduate School of Medicine, Akita-shi, Akita, 010-8543, Japan

    Yuki Moritoki

  3. Fourth Department of Internal Medicine, Teikyo University Mizonokuchi Hospital, Kawasaki-shi, Kanagawa, 213-8507, Japan

    Kentaro Kikuchi

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  1. Hayato Baba
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  2. Ayumi Sugitani
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Correspondence to Koichi Tsuneyama .

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  1. Department of Pathology, Shizuoka Cancer Center, Shizuoka, Japan

    Yasuni Nakanuma

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Baba, H. et al. (2017). Immunopathology of Bile Duct Lesions of Primary Biliary Cirrhosis. In: Nakanuma, Y. (eds) Pathology of the Bile Duct. Springer, Singapore. https://doi.org/10.1007/978-981-10-3500-5_6

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  • DOI: https://doi.org/10.1007/978-981-10-3500-5_6

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