primary billiard cirrhosis

Question 1

which antibodies are associated with this autoimmune condition?

Answer 1

• antimitochondrial antibodies- present in over 90% of cases and is a marker which has specificity
• anti-nuclear antibodies- 50% of cases

Question 2

what do the bloods look like for billiary cirrhosis?

Answer 2

• Alkaline phososphatase is first liver enzyme to be raised (as with most obstructive pathology)
• Other liver enzymes and bilirubin are raised in later disease
• ESR raised
• IgM raised

Question 3

what further investigations are used for diagnosis?

Answer 3

liver biopsy

Question 4

what is the management for PBC?

Answer 4

• 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- recurrence in 20% of cases

Question 5

what are the associations for PBC?

Answer 5

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

Question 6

which vitamins are lipid soluble?

Answer 6

AEDK

Question 7

why would you do MRCP in these patients?

Answer 7

• to exclude primary sclerosing cholangitis-> which will have beads on a string like appearance on MRCP
• to exclude any masses causing obstruction

Question 8

what is primary billiard cirrhosis?

Answer 8

Primary biliary cholangitis (previously primary biliary cirrhosis) is an autoimmune condition causing scarring and inflammation of the bile ducts, eventually leading to liver cirrhosis.