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Cirrhosis, Primary Biliary

Tara M. Kennedy, MD, Karl T. Clebak, MD, FAAFP and Megan H. Mendez Miller, DO Reviewed 06/2018
 


BASICS

DESCRIPTION

  • A chronic and progressive T-cell–mediated liver disease characterized by granulomatous destruction of intrahepatic bile ducts (1)

  • If untreated, primary biliary cirrhosis (PBC) can lea...

DIAGNOSIS

HISTORY

  • >60% of patients are asymptomatic at diagnosis.

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

TREATMENT

MEDICATION

First Line

  • Ursodeoxycholic acid (UDCA) is the only FDA-approved treatment for PBC and is associated with biochemical and histologic benefits. It does not affect symptoms such as fat...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Vaccinate patients against hepatitis A and hepatitis B (if not done previously).

  • Decrease alcohol intake, optimize weight management, promote tobacco cessation, an...

REFERENCES

1
Carey EJ, Ali AH, Lindor DL. Primary biliary cirrhosis. Lancet.  2015;386:1565–1575. {L-End} [View Abstract on OvidMedline]
2
Reshetnyak VI. Primary biliary cirrhosis: clinical and lab...

ADDITIONAL READING

Jacob DA, Bahra M, Schmidt SC, et al. Mayo risk score for primary biliary cirrhosis: a useful tool for the prediction of course after liver transplantation? Ann Transplant.  20...

CODES

ICD10

K74.3 Primary biliary cirrhosis 

ICD9

571.6 Biliary cirrhosis 

SNOMED

31712002 Primary biliary cirrhosis (disorder) 

CLINICAL PEARLS

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

  • Exclude other causes of cholestatic liver disease. Perform imaging (ultrasound, CT s...

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