primary biliary cirrhosis

Question 1

Define primary biliary cirrhosis?

Answer 1

chronic inflammatory liver disease involving progressive destruction of intrahepatic bile ducts ( smaller bile ducts that drain liver) leading to cholestasis and ultimately cirrhhosis

Question 2

outline the aetiology/ risk factors for primary biliary cirrhosis?

Answer 2

UNKNOWN

Likely to be autoimmune

Characterised by chronic autoimmune granulomatous inflammation which leads to fibrosis, cirrhosis and portal hypertension.

Genetic and environmental factors are involved

An environmental trigger may cause bile duct epithelial injury, which then leads to a T-cell mediated autoimmune response directed against bile duct epithelial cells

Question 3

summarise the epidemiology of primary biliary cirrhosis?

Answer 3

Mainly affects middle-aged women(9:1 female: male)

Question 4

what are the presenting symptoms of primary biliary cirrhosis?

Answer 4

May be an incidental findingon blood tests (inc alk phos)

Insidious onset with vague symptoms such as:

• Fatigue
• Weight loss
• Pruritis

Rarely, it may cause discomfort in the RUQ

May present with a complication of liver decompensation (e.g. jaundice, ascites, variceal haemorrhage)

May present with symptoms of associated conditions (e.g. Sjogren’s syndrome, arthritis, Raynaud’s phenomenon)

Question 5

What are the signs of primary biliary cirrhosis on physical examination?

Answer 5

Early - may be no signs

Late:

• Jaundice
• Skin pigmentation
• Scratch marks
• Xanthomas (secondary to hypercholesterolaemiawhich is a feature of cholestasis)
• Hepatosplenomegaly
• Ascites
• Signs of chronic liver disease

Question 6

what are the appopriate investigations for primary biliary cirrhsosis?

Answer 6

bloods

ultrasound

liver biopsy

Question 7

describe the bloods for primary biliary cirrhosis?

Answer 7

LFT:

• High ALP + GGT
• Bilirubin may be high or normal
• ALT and AST are normal initially but will increase as the disease progresses and cirrhosis develops
• Low albumin
• High prothrombin time

Clotting: prolongation of PT

Typical features of PBC:

• Antimitochondrial antibodies (typical feature of PBC) – AMAs are the hallmarks of PBC!!
• High IgM
• High Cholesterol

TFTs - because PBC is associated with autoimmune thyroid disease

Question 8

why is an ultrasound needed to investigate primary biliary cirrhosis?

Answer 8

Exclude extrahepatic biliary obstruction (e.g. gallstones)

Question 9

what is seen on the liver biospy in primary biliary cirrhosis?

Answer 9

• chronic inflammatory cells and granulomas around the intrahepatic bile ducts, destruction of bile ducts, fibrosis and regenerating nodules of hepatocytes

Question 10

what is the treatment for primary biliary cirrhosis?

Answer 10

Ursodeoxycholic acid ( exogenous bile salts)

Cholestyramine is used to alleviate pruritus but must be given at least 2 hours apart from ursodeoxycholic acid

Ultimately patients may need transplantation.

Statins are not generally used in PBC. Ursodeoxycholic acid lowers lipids and statins can obviously affect liver function therefore they do not form part of the initial treament.

Question 11

what are the differences between primary biliary cirrhosis and primary sclerosing cholangitis?

Answer 11