Primary Biliary Cholangitis

Question 1

What is primary biliary cholangitis and cirrhosis?

Answer 1

PBC
an autoimmune disease where T cells attack the cells that line the bile ducts in the liver.

antimitochondrial antibodies (AMAs). exist to get foreign mitochondria but there is molecular mimicry for proteins on biliary cells which has been caused due to infection/chemical trigger.

obstruction of bile (cholestasis) and bile leaks into the interstitial space and blood and liver cells (backpressure)

*symptoms similar to cholestasis and obstructive jaundice

advanced= cirrhosis

Question 2

PBC symptoms

Answer 2

Fatigue
Pruritus
GI disturbance and abdominal pain
Jaundice
Pale stools
Xanthoma and xanthelasma
Signs of cirrhosis and failure (e.g. ascites, splenomegaly, spider naevi)

• jaundice into blood (conjugated) (pruritis)
• xanthomas cholesterol in the blood)
• joint pain and arthropathy

raised ALP and GGT

tx: ursodeoxycholic acid and cholestyramine. (reduces cholesterol)

Question 3

secondary BC

Answer 3

similar complications
due to a tumour
no AMA’s

Question 4

PBS investigations

Answer 4

1. blood: LFT ALP raised
2. autoantibodies: antimitochondrial, IgM
3. ESR
4. liver biopsy

Question 5

PBS treatment

Answer 5

1. Ursodeoxycholic acid reduces the intestinal absorption of cholesterol
2. Cholestyramine is a bile acid sequestrate in that it binds to bile acids to prevent absorption in the gut and can help with pruritus due to raised bile acids
3. liver transplant in end stage
4. immunosuppression (steroids)