Primary Sclerosing Cholangitis

Question 1

Definition

Answer 1

Immune-mediated chronic liver disease that is characterised by inflammation, fibrosis and destruction of intrahepatic and/or extrahepatic bile ducts
the damage to bile ducts leads to:
- Cholestasis
- Bile and toxin build up in the liver
- Bile duct strictures
All of the above contribute to liver fibrosis, cirrhosis and progression to end-stage liver disease

Question 2

Epidemiology

Answer 2

Male
Age 40-50

Question 3

Risk factors

Answer 3

History of inflammatory bowel disease: usually UC
Family history
Genetic pre-disposition

Question 4

Aetiology

Answer 4

The cause is mostly unclear although there is likely to be combination of:
- genetic (HLA)
- autoimmune
- intestinal microbiome
- environmental factors
There is established association with UC

Question 5

Pathophysiology

Answer 5

Characterised by fibrosis or hardening (sclerosis) + inflammation of the intra and extrahepatic bile ducts
Some areas of bile duct tightened (due to fibrosis) while some areas are dilated = BEADED APPEARANCE ON ERCP
The strictures obstruct bile flow into intestines
- chronic bile obstruction can lead to liver inflammation (hepatitis), fibrosis, and cirrhosis
Damage to bile ducts leads to:
- Cholestasis
- Bile and toxin build up in liver
- Bile duct strictures
Too much fibrosis of intra-hepatic ducts = press on portal vein = portal htn = blood pushed back into liver + spleen = hepatosplenomegaly

Question 6

Signs

Answer 6

Jaundice + Pruritus
Signs of PSC complications:
Ascending cholangitis: Charcot’s triad
Chronic liver disease: rare at first presentation, e.g. ascites or encephalopathy

Question 7

Symptoms

Answer 7

Chronic RUQ pain
Fatigue
Itching
Symptoms of underlying inflammatory bowel disease:
- bloody stools
- tenesmus
- diarrhoea
- steatorrhea

Question 8

Diagnosis

Answer 8

Bloods
- elevated ALP/GGT (VERY HIGH) = Cholestatic picture
- elevated conjugated bilirubin ]
- ALT + AST high
P-ANCA positive (not specific)
AMA positive
Low albumin
GOLD STANDARD = MRCP
- show beaded appearance

Question 9

Treatment

Answer 9

Asymptomatic
FIRST LINE = observation + Life style changes
- Low alcohol
- Healthy diet
Cholestyramine = bile acid sequestrate = help itching
Fat soluble ADEK
ESLD: Liver transplantation: indicated when expected survival after transplantation exceeds that predicted without transplantation

Question 10

Complications

Answer 10

Cholangitis
Biliary strictures
Choledocholithiasis
Metabolic bone disease: osteoporosis
End stage liver disease: cirrhosis, portal hypertension and liver failure
cholangiocarcinoma
HCC
Colorectal carcinoma