Primary Biliary Cirrhosis

Question 1

What is Primary Biliary Cirrhosis?

Answer 1

Chronic, inflammatory liver disease involving progressive destruction of the intrahepatic bile ducts, leading to cholestasis.

Question 2

Which population is most likely to be affected by PBC?

Answer 2

Middle aged women

Question 3

Outline the pathology behind PBC

Answer 3

Autoimmune – think M&M: AMA autoantibodies, eventally IgM-mediated destruction.

Chronic granulomatuous infiltration of intrahepatic bile ducts with inflammation, fibrosis and eventually cirrhosis.

Question 4

How does PBC present?

Answer 4

Lethargy + pruritus

May present with later signs of liver disease (CAVE: Coagulopathy, Ascites, Varices, Encephalopathy)

Pain/tenderness not common.

Question 5

What investigations would you do in PBC?

Answer 5

LFTs – obstructive

Liver screen - ↑AMA

Imaging - USS abdo shows non-dilated biliary tree (excludes extra-hepatic obstruction as cause)

Question 6

How would you treat PBC?

Answer 6

Ursodeoxycholic acid – decreases toxicity of retained bile acids

Colestyramine/rifampicin/naloxone used for pruritus

Supplement vitamins A,D,E,K
Alleviate risk of osteoporosis with calcium and bisphosphonates

If disease is advanced/decompensated  put on list for liver transplant.

Question 7

What are some complications of PBC?

Answer 7

Liver cirrhosis + HCC, jaundice, CAVE, osteoporosis

Question 8

What is the prognosis for PBC?

Answer 8

Median survival 10y after diagnosis

PBC can recur in transplanted liver.