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Diagnosis and UDCA Treatment of Primary Biliary Cirrhosis

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Autoimmune Liver Diseases

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Abstract

The diagnosis of PBC is largely based on three features: persistent elevation of cholestatic liver enzymes (ALP and/or GGT), detectable anti-mitochondrial autoantibodies (AMAs), and liver histology. The clinical guidelines from the AASLD, EASL, and Japan agree that the diagnosis of PBC can be made in patients who met two criteria from the above three clinical features. In atypical cases in whom serum AMAs are negative or elevation of AST and/or ALT is remarkable a liver biopsy is mandatory. The role of ursodeoxycholic acid (UDCA) for treatment of PBC is well established by several clinical trials, and UDCA is the only approved drug for PBC worldwide. There are, however, some patients with PBC who are refractory to UDCA treatment, and the prognosis is shown to be worse in these cases. The second-line treatment is strongly warranted and bezafibrate is a robust candidate for this, although long-term effect of bezafibrate has not been demonstrated.

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Authors and Affiliations

  1. Department of Medicine, Teikyo University School of Medicine, 2-11-1, Kaga, Itabashi-ku, Tokyo, 173-8605, Japan

    Atsushi Tanaka

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  1. Atsushi Tanaka
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Correspondence to Atsushi Tanaka .

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Editors and Affiliations

  1. Dept of Gastroenterology & Rheumatology, Fukushima Medical University of Medicine, Fukushima, Japan

    Hiromasa Ohira

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Tanaka, A. (2014). Diagnosis and UDCA Treatment of Primary Biliary Cirrhosis. In: Ohira, H. (eds) Autoimmune Liver Diseases. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54789-1_18

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  • DOI: https://doi.org/10.1007/978-4-431-54789-1_18

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  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-54788-4

  • Online ISBN: 978-4-431-54789-1

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