Primary Sclerosing Cholangitis, Primary Biliary Cholangitis

Question 1

What is PSC?

Answer 1

intrahepatic or extrahepatic ducts become strictured and fibrotic
Causes obstruction to the flow of bile out of the liver and into the intestines

Sclerosis = stiffening and hardening of the bile ducts Cholangitis = inflammation of the bile ducts

Chronic bile obstruction eventually leads to liver inflammation (hepatitis), fibrosis and cirrhosis

Question 2

Risk factors for PSC

Answer 2

Male
Aged 30-40
Ulcerative Colitis
Family History

Question 3

Presentation of PSC (5)

Answer 3

Jaundice
Chronic right upper quadrant pain
Pruritus
Fatigue
Hepatomegaly

Question 4

What is seen on the LFTs?

Answer 4

“cholestatic” picture

Alkaline phosphatase is the most deranged LFT and may be the only abnormality at first

Bilirubin may rise as the strictures become more severe and prevents bilirubin from being excreted through bile duct
Other LFTs (i.e. transaminases: ALT and AST) can also be deranged, particularly as the disease progresses to hepatitis.

Question 5

How is PSC diagnoased?

Answer 5

MRCP

Question 6

Associations and complications of PSC

Answer 6

Acute bacterial cholangitis
Cholangiocarcinoma develops in 10-20% of cases
Colorectal cancer
Cirrhosis and liver failure
Biliary strictures
Fat soluble vitamin deficiencies

Question 7

Management of PSC

Answer 7

Liver transplant

ERCP can be used to dilate and stent any strictures
Ursodeoxycholic acid is used and may slow disease progression
Colestyramine binds to bile acids to prevent absorption in the gut - can help with pruritus due to raised bile acids

Question 8

What is PBC?

Answer 8

Immune system attacks the small bile ducts
First parts to be affected are the intralobar ducts - Canals of Hering

Back-pressure of the bile obstruction and l disease process leads to fibrosis, cirrhosis and liver failure

Question 9

Presentation of PBC (7)

Answer 9

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

Question 10

Associations with PBC patients

Answer 10

Middle aged women
Other autoimmune diseases (e.g. thyroid, coeliac)
Rheumatoid conditions (e.g. systemic sclerosis, Sjogrens and rheumatoid arthritis)

Question 11

diagnosis of PBC

Answer 11

Alkaline phososphatase is first liver enzyme to be raised
Other liver enzymes and bilirubin are raised in later disease

Anti-mitochondrial antibodies is the most specific to PBC and forms part of the diagnostic criteria
Anti-nuclear antibodies are present in about 35% of patients

ESR raised
IgM raised

Question 12

Treatment of PBC

Answer 12

Ursodeoxycholic acid reduces the intestinal absorption of cholesterol
Colestyramine 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

Liver transplant in end stage liver disease
Immunosuppression (e.g. with steroids) is considered in some patients

Question 13

Other complications with PBC (7)

Answer 13

Symptomatic pruritus
Fatigue
Steatorrhoea (greasy stools due to lack of bile salts to digest fats)
Distal renal tubular acidosis
Hypothyroidism
Osteoporosis
Hepatocellular carcinoma