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Primary Biliary Cirrhosis (PBC) (Risk factors (f:m = 9:1, :heavy_plus_sign…
Primary Biliary Cirrhosis (PBC)
Pathology
Intralobular bile ducts damaged
By chronic autoimmune granulomatous inflammation
Leads to fibrosis, cirrhosis, portal hypertension
Causing cholestasis
Causes
unknown environmental triggers
plus genetic predisposition
leading to loss of immune tolerance to self mitochondrial proteins
AMA antimitochondrial Ab are present
Risk factors
f:m = 9:1
:heavy_plus_sign:family history
many UTIs
smoking
past pregnancy
other autoimmune diseases
:arrow_up:use of hair polish and nail dye
Presentation
Typically at 50 years
Often aysmptomatic and diagnosed after finding raised ALP on routine bloods
These may precede jaundice by years
lethargy
sleepiness
pruritus
Jaundice
Skin pigmentation
Xanthelasma
Xanthomata
Complications
Cirrhosis
osteoporosis
osteomalacia
as malapsorption of fat soluble vitamins (ADEK) due to cholestasis and decreased bilirubin in the gut bilirubin, causes osteomalacia and coagulopathy
coagulopathy
HCC
check AFP twice yearly
Ix
Bloods
:arrow_up:ALP
:arrow_up:GGT
Mildly :arrow_up:ALT & AST
Late disease
:arrow_up:bilirubin
:arrow_down:albumin
:arrow_up:immmunoglobulins espcially IGM
:arrow_up: or :left_right_arrow:TSH & cholesterol
USS
excludes extrahepatic cholestasis
Biopsy
not needed unless excluding
Drug induced cholestasis
Hepatic sarcoidosis
TB
parasites/schistosomiasis
Treatment
Prutitus: cholestyramine
Diarrhoea: codeine phosphate
Osteoporosis prevention
Vitamin ADEK fat soulube vitmain
high dose ursodeoxycholic acid
can reduce ALP
Can reduce bilirubin
monitoring
LFT
USS
AFP
Liver transplant
For end stage
Intractable pruritus
Can recur in graft 17% after 5 years
Prognosis
once jaundice develops, survival is <2 years without transplant