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Cholangitis, Primary Biliary

Maisa E Elbadawi, MD and Karim T Osman, MD Reviewed 04/2024
 


BASICS

DESCRIPTION

  • Primary Biliary Cholangitis (PBC) is an autoimmune disease characterized by chronic progressive destruction of small intrahepatic bile ducts with portal inflammation resulting in fib...

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.

  • The optimal dose is 13 to 15 mg/kg/day and no dose escala...

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
Shah RA, Kowdley KV. Current and potential treatments for prima...

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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