Primary Biliary Cirrhosis Flashcards
Define Primary Biliary Cirrhosis (PBC)?
A chronic inflammatory liver disease involving progressive destruction of intrahepatic bile ducts, leading to cholestasis, and cirrhosis
What is the aetiology of PBC?
Likely to be autoimmune
Genetic and environmental factors are involved
An environmental trigger may cause bile duct epitherlial injury, which then leads to a T-cell mediated autoimmune response directed against bile duct epithelial cells
What is the epidemiology of PBC?
Mainly affects middle-aged women (9:1 female: male)
What are the presenting symptoms of PBC?
May be an incidental finding on blood tests
Insidious onset with vague symptoms such as:
- Fatigue
- Weight Loss
- Pruritis
What are some of the rarer presenting symptoms of PBC?
It may cause discomfort in the RUQ
Complication of liver decompensation
Symptoms of Associated conditions
What are some complications of liver decompensation?
Jaundice
Ascites
Variceal haemorrhage
What are some of the associated conditions of PBC?
Sjogren’s Syndrome
Arthritis
Raynaud’s phenomenon
What are the early signs of PBC on physical examination?
May be no signs
What are the Late signs of PBC on physical examination?
Jaundice Skin pigmentation Scratch marks Xanthomas (secondary to hypercholesterolaemia) Hepatomegaly Ascites Signs of Chronic liver disease
What bloods do we do for PBC?
LFT
Clotting
Typical features of PBC
TFTs
What do we look for specifially on LFTs for PBC?
High ALP + GGT
Bilirubin may be high or normal
ALT and AST are normal initially but will increase as the disease progresses and cirrhosis develops
What do we look for on clotting tests for PBC?
Prolongation of PT
What are the typical features of PBC that we see on Bloods?
Antimitochrondrial antibodies (typical feature of PBC)
High IgM
High cholesterol
Why do we do TFTs for PBC?
PBC is associated with autoimmune thyroid disease
Why do we do an US for PBC?
Exclude extrahepatic biliary obstruction (e.g. gallstones)
