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Autoimmune Liver Diseases: Primary Biliary Cholangitis

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Liver Disorders

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Abstract

Primary biliary cirrhosis (PBC) is an autoimmune disease characterized by cholestasis and destruction of the small intralobular bile ducts. It affects mainly middle-aged women. The antimitochondrial antibody (AMA) is the serological hallmark of the disease, present in ~95 % of patients. Fatigue and pruritus are the most common symptoms of PBC. They are often mild, but can be very debilitating. PBC can progress to end-stage liver disease, cirrhosis, and its consequent complications. PBC is an important risk factor for hepatocellular carcinoma. Ursodeoxycholic acid (UDCA) is the only drug approved for the treatment of PBC. The survival of patients with early-stage PBC who have complete response to UDCA mirrors that of the general population. PBC patients with incomplete response to UDCA are at high risk for adverse events. Clinical trials of newer agents are underway.

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Authors and Affiliations

  1. Division of Gastroenterology and Hepatology, Mayo Clinic, Phoenix, AZ, USA

    Ahmad H. Ali M.B.B.S., Elizabeth J. Carey M.D. & Keith D. Lindor M.D.

  2. College of Health Solutions, Arizona State University, 550 North 3rd Street, Phoenix, AZ, 85004, USA

    Keith D. Lindor M.D.

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  1. Ahmad H. Ali M.B.B.S.
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  2. Elizabeth J. Carey M.D.
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  3. Keith D. Lindor M.D.
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Correspondence to Keith D. Lindor M.D. .

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Editors and Affiliations

  1. Division of Gastroenterology and Hepatology, Medical College of Wisconsin , Milwaukee, Wisconsin, USA

    Kia Saeian

  2. Division of Gastroenterology and Hepatology, Medicine Medical College of Wisconsin , Milwaukee, Wisconsin, USA

    Reza Shaker

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Ali, A.H., Carey, E.J., Lindor, K.D. (2017). Autoimmune Liver Diseases: Primary Biliary Cholangitis. In: Saeian, K., Shaker, R. (eds) Liver Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-30103-7_16

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