Ulcerative Colitis

Question 1

What are the reference ranges for LFTs?

Answer 1

• ALT < 40
• AlkPhos 30 - 130
• bilirubin 2 - 21
• albumin 35 - 50

Question 2

What would a raised ALT suggest?

Answer 2

raised ALT is a marker for heptocyte injury

Question 3

What would a raised alkaline phosphastase suggest?

Answer 3

raised AlkPhos is a marker for bile duct injury

Question 4

What is involved in the non-invasive liver screen?

Answer 4

• ANCA
• IgG / IgA / IgM
• autoantibodies
• often accompanied by an ultrasound scan of the liver

Question 5

What are the 2 main types of chronic biliary diseases?

Answer 5

1. primary biliary cholangitis (PBC)
2. primary sclerosing cholangitis (PSC)

Question 6

What abnormal features should be looked for when looking at the histology of the liver?

Answer 6

• portal inflammation
• “onion skin” periductal fibrosis
• ductopenia & fibro-obliterative scars
  
  • scars represent where bile ducts would have been, but have now disappeared
• scattered areas of parenchymal inflammation
• shikata stain - periportal copper associated protein deposition
  
  • ​bile salts depositied within hepatocytes that cannot escape due to bile duct injury

Question 7

What would ductopenia look like?

Answer 7

there would be a portal tract present, but without any ducts

Question 9

What are important things to note in terms of the histology for primary sclerosing cholangitis?

Answer 9

• portal changes often patchy, especially in early stages
  
  • a “normal biopsy” does not exclude the disease
• progression with cholestasis, fibrosis & eventually cirrhosis
• increased risk of developing dysplasia in bile ducts (BiIN) which can progress to cholangiocarcinoma

Question 10

What is primary sclerosing cholangitis?

What is it associated with?

Answer 10

slowly (& unpredictably) progressive chronic cholestatic liver injury with fibrosing destruction of bile ducts - either intrahepatic or extrahepatic

it is an immune mediated disorder

it has a strong association with inflammatory bowel disease (usually ulcerative colitis)

genetic susceptibility - HLA-A1-B8-DR3 haplotype

Question 11

Who tends to be affected by primary sclerosing cholangitis?

Answer 11

it is more common in males and 70% of cases are in males

the median age of presentation is 40 years

it can affect any age, including children

Question 12

What are the clinical features of primary sclerosing cholangitis?

Answer 12

• the majority of patients are asymptomatic
  
  • ​routine blood tests will show LFT abnormalities
• fluctuating pruritis
• fatigue
• jaundice
• recurrent bacteria cholangitis (RUQ pain, fever & jaundice)
• eventually features of cirrhosis & portal hypertension

Question 13

What are the investigations and results in someone with primary sclerosing cholangitis?

Answer 13

Bloods:

• LFTs show a mixture of hepatitic (raised ALT) & cholestatic picture (raised AlkP & bili)
• ANCA +ve

Imaging:

• US - exclude focal lesions e.g. cholangiocarcinoma
• MRCP / ERCP - multiple biliary strictures & areas of dilatation

liver biopsy

Question 14

What are the management options for PSC?

What are the management options aimed at?

Answer 14

aimed at treating symptoms and slowing disease progression

ursodeoxycholic acid (UDCA):

• bile salt analogue
• symptom control and slow progression of disease

immunosuppression:

• steroids, azathioprine, methotrexate

biliary balloon dilatation / stenting at ERCP

• used if there are areas of stenosis within the larger ducts

Question 15

What is the prognosis of PSC like?

What are the risks associated with this condition?

Answer 15

• slowly progressive, eventually leading to cirrhosis and end stage liver failure
• average survival 12-17 years from diagnosis (without transplant)
• increased risk of developing cholangiocarcinoma
• liver transplant - risk of recurrence