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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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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