Primary Biliary Cirrhosis

Question 1

Hallmark

Answer 1

anti-mitochondrial antibodies (AMAs)

Question 2

cause

Answer 2

unknown environmental triggers + genetic predisposition –> loss of immune tolerance to self-mitochonidal proteins

Question 3

suggested pathogenesis

Answer 3

interlobular bile ducts damaged by chronic autoimmune granulomatous inflammation

–> cholestasis

may –> fibrosis, cirrhosis and portal HTN

Question 4

Ductopenia

Answer 4

Portal vein, hepatic artery, but no bile duct.

Immune damage directed to the small bile ducts

Question 5

epidemilogy

Answer 5

~50yrs

90% are women!

Question 6

symptoms

Answer 6

often incidental finding of raised alk phos on routine LFT

lethargy, sleepiness and pruritus may precede jaundice by years

Question 7

signs

Answer 7

jaundice, skin pigmentation, hepatosplenomegaly

Question 8

complications

Answer 8

cirrhosis
osteoporosis

malab of fat-soluble vitamins due to cholestasis and reduced billirubin in gut lumen –> osteomalacia and coagulopathy

Question 9

blood results

Answer 9

raised alk phos, yGT
mildly raised AST & ALT
raised serum cholesterol

Question 10

what does ultrasound exclude?

Answer 10

extra hepatic cholestasis

Question 11

Tx of symptoms

Answer 11

itching - colestyramine
diarrhoea - codeine phosphate
osteoporosis prevention - bisphosphonates
fatigue - modafinil, vitsADEK supplements

Question 12

what is the only routine Tx?

Answer 12

urodeoxycholic acid

improves liver enzymes, bilirubin

Question 13

what improves biochemical and histological disease but has side effects?

Answer 13

steroids

Question 14

whats the prognosis once jaundice develops?

Answer 14

2 yrs without transplantation