PBC Flashcards
What is PBC?
chronic inflammatory liver disease involving progressive destruction of INTRAhepatic bile ducts, leading to cholestasis, fibrosis + cirrhosis
Describe the aetiology of PBC
UNKNOWN
Likely AI (Genetic + environmental factors)
Describe the epidemiology of PBC
F > M (9:1)
Often middle aged women
List 4 conditions associated with PBC
Sjogren’s syndrome (in up to 80%)
RhA
Systemic sclerosis
Thyroid disease
List 6 clinical features of PBC
Fatigue
Pruritis
Sx of cholestasis: pale stool, dark urine, jaundice
Hyperpigmentation
Xanthelasmas, Xanthomata
Clubbing
Hepatomegaly
Which bloods should be taken in suspected PBC?
LFTs, Albumin
FBC
Lipid panel
Autoantibodies
Immunoglobulins
Describe the LFTs in PBC
high ALP
high GGT
high BR
mild transaminitis/ normal ALT + AST
low albumin
What lipids are seen in PBC?
Hypercholesterolaemia due to altered lipoprotein metabolism
Which autoantibodies and immunoglobulins are seen in PBC?
Anti-MITOCHONDRIAL antibodies (>95%)
ANA (~50%)
High IgM
Why perform an ultrasound/ MRCP in PBC?
Exclude extrahepatic biliary obstruction (e.g. gallstones)
When is liver biopsy performed in PBC?
Most DONT require biopsy for dx
Performed to confirm dx if no characteristic antibodies or r/o ddx
What may be seen on liver biopsy in PBC?
Non-supparative cholangitis + destruction of small + medium bile ducts
What ducts are affected in PBC?
INTRAHEPATIC
Describe medical management of PBC
Ursodeoxycholic acid: slows progression + improves Sx
Cholestyramine reduces pruritus
Fat-soluble vitamin supplementation
What is the definitive management of PBC? When is this indicated?
Liver transplantation
If liver cirrhosis, BR >100
