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Diseases of the Liver: Primary Biliary Cholangitis

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Abstract

Primary biliary cholangitis (PBC) is a chronic, autoimmune disease that results in destruction of intrahepatic bile ducts, ultimately leading to cirrhosis and liver failure. PBC is predominantly diagnosed in females between 40 and 60 years of age. While rare, the incidence and prevalence of PBC is increasing. Characteristic serologic findings include elevated alkaline phosphatase (ALP) and antimitochondrial antibody (AMA) levels. Clinical manifestations of PBC include fatigue, pruritus, metabolic bone disease, hypercholesterolemia, malabsorption, anemia, and deficiencies of fat-soluble vitamins. Concomitant autoimmune diseases, including hypothyroidism, are often present. Ursodeoxycholic acid (UDCA) is the mainstay of therapy and has been shown to improve transplant-free survival. The use of adjunct therapies is becoming more prevalent, particularly in patients with an inadequate response to UDCA. Patients with advanced- or end-stage liver disease (ESLD) should be considered for liver transplantation (LT), which is associated with excellent graft and patient outcomes.

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Abbreviations

AIH:

Autoimmune hepatitis

ALP:

Alkaline phosphatase

ALT:

Alanine aminotransferase

AMA:

Antimitochondrial antibody

ANA:

Antinuclear antibody

AST:

Aspartate aminotransferase

BEC:

Biliary epithelial cell

BMD:

Bone mineral density

ESLD:

End-stage liver disease

FDA:

Food and Drug Administration

FXR:

Farnesoid X receptor

GGT:

Gamma-glutamyl transferase

HLA:

Human leukocyte antigen

IL:

Interleukin

LT:

Liver transplantation

MELD:

Model for End-Stage Liver Disease

OCA:

Obeticholic acid

PBC:

Primary biliary cholangitis

PDC:

Pyruvate dehydrogenase complex

PPAR:

Peroxisome proliferator-activated receptor

SMAB:

Smooth muscle antibody

UDCA:

Ursodeoxycholic acid

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Acknowledgment

We would like to acknowledge Dr. Rashmi Agni from the University of Wisconsin Department of Pathology and Laboratory Medicine for providing the pathology images for this chapter.

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Authors and Affiliations

  1. Division of Gastroenterology and Hepatology, Department of Medicine, University of Wisconsin School of Medicine and Public Health and Wm S Middleton VAMC, Madison, WI, USA

    Paulina K. Phillips & Adnan Said

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  1. Paulina K. Phillips
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  2. Adnan Said
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Correspondence to Adnan Said .

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Editors and Affiliations

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

    Poonam Beniwal-Patel

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

    Reza Shaker

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Phillips, P.K., Said, A. (2019). Diseases of the Liver: Primary Biliary Cholangitis. In: Beniwal-Patel, P., Shaker, R. (eds) Gastrointestinal and Liver Disorders in Women’s Health . Springer, Cham. https://doi.org/10.1007/978-3-030-25626-5_7

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  • DOI: https://doi.org/10.1007/978-3-030-25626-5_7

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