Definition

[1]
Primary Biliary Cirrhosis (also PBC) is a disease where the intrahepatic bile ducts are damaged due to chronic portal inflammation. Fibrosis develops and eventually cirrhosis. The disease is thought to be of autoimmune origin.

Incidence

[2]
Lifetime prevalence of 35 per 100 000

Age

45 to 50

Gender

Up to 10 times more likely in females

Risk Factors

[3]

Signs and Symptoms

[4]

Pathophysiology

[5]
Primary biliary cirrhosis (PBC) is progressive damage to epithelial cells of the intrahepatic biliary tree. There is portal tract inflammation often in the context of anti-mitochondrial antibodies suggesting that the disease is of autoimmune origin. Eventually there is complete loss of small intrahepatic ducts leading to cholestasis and bile salt retention. This causes damage within the liver which in turn leads to further bile duct loss. The liver damage causes fibrosis which can ultimately progress to cirrhosis.

Investigations

[6]

Bloods

Imaging

  • Ultrasound - rule out obstruction (see gallstones, cancer)

Other

  • Liver biopsy (if there is uncertainty) - bile duct lesions and granuloma formation

Diagnosis

[7]
Diagnosis is made clinically with a context of autoimmune antibodies. Imaging must be used to rule out obstructive causes before making the diagnosis.

Differentials to rule out

Treatment

[8]

Non-Pharmacological

  • Lifestyle modifications due to fatigue

Pharmacological

  • Ursodeoxycholic acid (reduces symptoms of liver damage)
  • Immunosuppression with prednisolone

Surgical

  • Liver transplant

Management

[9]
Patients should be followed up for signs of improvement with treatment. If there is no significant improvement then patients will require a liver transplant.

Complications

[10]

Prognosis

[11]
Primary Biliary Cirrhosis is often a progressive disease even with treatment. Most patients die of non-liver related disease due to the age of onset. Primary Biliary Cirhosis doubles the chance of dying with end-stage liver disease, doubles all cause mortality and reduces quality of life due to fatigue and other symptoms.

See Also

BMJ Best Practice - Primary Biliary Cirrhosis
  1. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/344.html
  2. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/344.html
  3. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/344.html
  4. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/344.html
  5. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/344.html
  6. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/344.html
  7. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/344.html
  8. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/344.html
  9. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/344.html
  10. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/344.html
  11. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/344.html