FN: Primary Biliary Cirrhosis Flashcards
1
Q
Epi
A
Prev: 4/100,000
Sex: F»M = 9:1
Age: 50s
2
Q
Pathology
A
Intrahepatic bile duct destruction by chronic
granulomatous inflammation → cirrhosis
3
Q
Presentation PPBBCCS
A
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
Q
Associated Disease
A
Thyroid disease RA, Sjogrens, scleroderma Coeliac disease RTA Membranous GN
5
Q
Ix
A
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
Q
Rx symptomatic
A
Pruritus: colestyramine, naltrexone Diarrhoea: codeine phosphate
Osteoporosis: bisphosphonates
7
Q
Specific Mx
A
ADEK vitamins
Ursodeoxcholic acid: ↓LFTs but no effect on mortality or need for transplant
8
Q
Liver transplant
A
End-stage disease or intractable pruritus
Recurrence occurs in ~20% but doesn’t usually
→ graft failure.
9
Q
Prognosis
A
Once jaundice develops survival is <2yrs
