Primary Biliary Cirrhosis Flashcards Preview

Liver + GI > Primary Biliary Cirrhosis > Flashcards

Flashcards in Primary Biliary Cirrhosis Deck (14)
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1

Hallmark

anti-mitochondrial antibodies (AMAs)

2

cause

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

3

suggested pathogenesis

interlobular bile ducts damaged by chronic autoimmune granulomatous inflammation

--> cholestasis

may --> fibrosis, cirrhosis and portal HTN

4

Ductopenia

Portal vein, hepatic artery, but no bile duct.

Immune damage directed to the small bile ducts

5

epidemilogy

~50yrs
90% are women!

6

symptoms

often incidental finding of raised alk phos on routine LFT

lethargy, sleepiness and pruritus may precede jaundice by years

7

signs

jaundice, skin pigmentation, hepatosplenomegaly

8

complications

cirrhosis
osteoporosis

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

9

blood results

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

10

what does ultrasound exclude?

extra hepatic cholestasis

11

Tx of symptoms

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

12

what is the only routine Tx?

urodeoxycholic acid
improves liver enzymes, bilirubin

13

what improves biochemical and histological disease but has side effects?

steroids

14

whats the prognosis once jaundice develops?

2 yrs without transplantation