primary biliary cirrhosis

Question 1

definition of primary biliary cirrhosis

Answer 1

chronic autoimmune granulomatous inflammatory liver disease

involving progressive destruction of intrahepatic/intralobular bile ducts

leading to cholestasis -> fibrosis and ultimately cirrhosis and portal hypertension

Question 2

aetiology of primary biliary cirrhosis

Answer 2

unknown

autoimmune likely

genetic (IL12A locus) and env suggested

env trigger (infection, chem, pollutants, xenobiotics or toxin) may = bile duct epithelial injury

in suseptible individuals -> T cell mediated autoimmune response directed at bile duct epithelial cells

loss of immune tolerance to self-mitochondrial proteins

Question 3

epidemiology of primary biliary cirrhosis

Answer 3

prev 10-20 in 100000 in UK

middle aged women

female more

typical age at presentation - 50yrs

Question 4

sx of primary biliary cirrhosis

Answer 4

incidental finding - high AlkPhos, high cholesterol

insidious onset - fatigue, weight loss, pruritus

discomfort in RUQ - rare

present with complication of liver decompensation eg jaundice, ascites, variceal haemorrhage

sx of associated conditions

Question 5

conditions associated with primary biliary cirrhosis

Answer 5

sjogren’s syndrome - dry eyes and mouth

arthritis

raynauds

Question 6

signs of primary biliary cirrhosis

Answer 6

early - may be none

late:

• jaundice
• skin pigmentation
• scratch marks
• xanthomas, xanthalasma, xanthomata - secondary to hypercholosteraemia
• hepatosplenomegaly
• ascites

signs of chronic liver disease - palmar erythema, clubbing, spinder naevi

Question 7

Ix for primary biliary cirrhosis

Answer 7

blood

US
liver biopsy

Question 8

blood results for primary biliary cirrhosis

Answer 8

LFT

• high ALP,
• high GGT,
• BR normal or high in late stage
• transaminase initially normal, increase with progression and development of cirrhosis
• low albumin in late disease

clotting - prolongation of PT

antimitochondrial Ab (AMA), high IgM and high cholesterol are typical

other autoAb may ocur at low levels

TFT - associated with autoimmune thyroid disease (TSH)

plasma ca

phosphate

25-hydroxyvit D

Question 9

US for primary biliary cirrhosis

Answer 9

exclude extrahepatic biliary obstruction eg by gallstones or strictures

Question 10

liver biopsy in primary biliary cirrhosis

Answer 10

chronic inflammatory cells and granulomas around the intrahepatic bile ducts

destruction of bile ducts

fibrosis

cirrhosis

regenerating nodules of hepatocytes

repeat while on treatment

(not usually necessary unless drug induced cholestasis or hepatic sarcoidosis need excluding)

Question 11

hallmark of primary biliary cirrhosis

Answer 11

AMA

Question 12

RF for primary biliary cirrhosis

Answer 12

+ve FH

many UTIs

smoking

past pregnancy

other autoimmune diseases

high use of nailpolish/hair dye

Question 13

Mx of PBC

Answer 13

1. ursodeoxycholic acid - modifies disease progression
   obeticholic acid instead if hepatic impairment
2. colestyramine for antipyurituc
3. lifestyle

if end stage -> liver transplant

Question 14

complications of PBC

Answer 14

hypercholesteraemia
osteoporosis
portal HTN secondary to cirrhosis
hepatocellular cancer