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Cirrhosis, Primary Biliary

Karim T Osman, MD, Joseph M Cappuccio, DO and Fredric D. Gordon, AGAF, FAASLD, MD Reviewed 06/2022
 


BASICS

DESCRIPTION

  • Primary Biliary Cholangitis (PBC) is a chronic autoimmune disease characterized by the destruction of intrahepatic cholangiocytes and progressive cholestasis. The diagnostic hallmark...

DIAGNOSIS

HISTORY

  • 20–60% of patients are asymptomatic at diagnosis  (1).

  • Often identified by incidental finding of elevated serum alkaline phosphatase (ALP), γ-glutamyl transpeptidase (GGTP), or total s...

TREATMENT

MEDICATION

First Line

  • UDCA improves transplant-free survival. When started early, overall survival approaches the general population's [A].

  • The optimal dose is 13 to 15 mg/kg/day. It does not a...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Vaccinate nonimmune patients against hepatitis A and hepatitis B.

  • Decrease alcohol intake and optimize weight management. Avoid hepatotoxic drugs.

  • Pregnant women ne...

REFERENCES

1
Lleo A, Marzorati S, Anaya J-M, et al. Primary biliary cirrhosis: a comprehensive overview. Hepatol Int. 2017;11(6):485–499.
2
Reshetnyak  VI. Primary biliary cirrhos...

ADDITIONAL READING

Colapietro F, Lleo A, Generali E. Antimitochondrial antibodies: from bench to bedside. Clin Rev Allergy Immunol. 2021:1–12. doi: 10.1007/s12016-021-08904-...

CODES

ICD10

K74.3 Primary biliary cirrhosis 

SNOMED

31712002 Primary biliary cirrhosis (disorder) 

CLINICAL PEARLS

  • Consider PBC in middle-aged women with unexplained pruritus or asymptomatic elevation of ALP.

  • AMA is positive in 95% of cases. Consider liver biopsy if AMA is negative or AST is >5 t...

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