FN: Primary Biliary Cirrhosis Flashcards Preview

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Flashcards in FN: Primary Biliary Cirrhosis Deck (9)
1

Epi

􏰀 Prev: 􏰂4/100,000
􏰀 Sex: F>>M = 9:1
􏰀 Age: 50s

2

Pathology

􏰀 Intrahepatic bile duct destruction by chronic
granulomatous inflammation → cirrhosis

3

Presentation PPBBCCS

􏰀 Often asympto and Dx incidentally (↑ALP)
􏰀 Jaundice occurs late
􏰀 Pruritus and fatigue
􏰀 Pigmentation of face
􏰀 Bones: osteoporosis, osteomalacia (↓ vit D)
􏰀 Big organs: HSM
􏰀 Cirrhosis and coagulopathy (↓ vit K)
􏰀 Cholesterol ↑: xanthelasma, xanthomata
􏰀 Steatorrhoea

4

Associated Disease

􏰀 Thyroid disease
􏰀 RA, Sjogrens, scleroderma
􏰀 Coeliac disease
􏰀 RTA
􏰀 Membranous GN

5

Ix

􏰀 LFTs: ↑↑ALP, ↑↑GGT, ↑AST/ALT
􏰁 Late: ↑BR, ↑PT, ↓albumin
􏰀 Abs: AMA+ (98%)
􏰀 ↑IgM
􏰀 ↑ cholesterol
􏰀 ±↑TSH
􏰀 US to exclude extra-hepatic cholestasis
􏰀 Liver biopsy: non-caseating granulomatous inflam

6

Rx symptomatic

􏰁 Pruritus: colestyramine, naltrexone 􏰁 Diarrhoea: codeine phosphate
􏰁 Osteoporosis: bisphosphonates

7

Specific Mx

􏰁 ADEK vitamins
􏰁 Ursodeoxcholic acid: ↓LFTs but no effect on mortality or need for transplant

8

Liver transplant

􏰁 End-stage disease or intractable pruritus
􏰁 Recurrence occurs in ~20% but doesn’t usually
→ graft failure.

9

Prognosis

􏰀 Once jaundice develops survival is <2yrs

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