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Primary biliary cirrhosis (PBC) (Risk factors (FH, Smoking, Other…
Primary biliary cirrhosis (PBC)
Definition
Autoimmune disease
affecting bile ducts
in the liver
Pathophysiology
Granulotomatous inflammation
in interlobular bile ducts
Inflammation causes cholestasis
Fibrosis, cirrhosis, portal HTN
Environmental trigger (pollutants, infection)
plus genetic predisposition
Epidemiology
Rare
F>M
Age 50y
Risk factors
FH
Smoking
Other autoimmune disease
Recurrent UTIs
Past pregnancy
Clinical
presentation
Asymptomatic
(incidental finding)
Symptomatic
Pruritus
Jaundice
Lethargy
Diagnosis
Examination
Abdo exam
Jaundice, excoriations, xanthelasma/xanthomata,
hepatosplenomegaly
Investigations
Bloods
FBC, CRP, U+Es, LFTs (high ALP/y-GT, mild AL/AST;
late disease high bilirubin, low Alb, elevated PT time)
Immunology (AMA +ve, high IgM)
Imaging
Abdo USS (exclude obstruction)
Biopsy
Often not needed (changes not uniform;
see formation of granulomas, cirrhosis)
Bedside
Obs (nil)
History
DH
Meds, allergies
FH
Autoimmune liver disease,
other autoimmune diseases
PMH
Other autoimmune disease
SH
Living arrangements, occupation,
smoking, alcohol
PC/HPC
Lethargy, pruritus
Management
Medical
Anti-pruritic
Indication: pruritus
E.g. colestyramine PO
Bile acid replacement
Indication: all patients
E.g. ursodeoxycholic acid (UDCA)
MOA: bile acid replacement
SE: weight gain
Vitamin replacement
Indication: all patients
E.g. fat-soluble vitamins ADEK
Surgical
Liver transplant
End-stage disease
Can get recurrence
Conservative
Information, advice, support
Lifestyle (alcohol, weight, smoking)
Monitoring (LFTs, USS, AFP)
Complications
Cirrhosis
Osteoporosis
HCC
Malabsorption of
fat-soluble vits (ADEK) leads
to osteomalacia, coagulopathy
Prognosis
<2y once jaundice
occurs if no transplant