Primary Biliary Cirrhosis Flashcards
1
Q
Define
A
- Intralobular bile ducts are damaged by chronic autoimmune granulomatous inflammation causing cholestasis which may lead to fibrosis, cirrhosis and portal HTN
- Slowly progressive, insidious onset, may extend over many decades
2
Q
Causes
A
UNKNOWN
Likely to be autoimmune
Genetic and environmental factors are involved
An environmental trigger may cause bile duct epithelial injury, which then leads to a T-cell mediated autoimmune response directed against bile duct epithelial cells
3
Q
Risk factors
A
↑risk with a FHx, many UTIs, smoking, past pregnancy, other AI disease, use of nail polish
4
Q
Epidemiology
A
Mainly affects middle-aged women (9:1 female: male)
5
Q
Symptoms
A
May be an incidental finding on blood tests
Insidious onset with vague symptoms such as:
- Fatigue
- Weight loss
- Pruritis
Rarely, it may cause discomfort in the RUQ
- May present with a complication of liver decompensation (e.g. jaundice, ascites, variceal haemorrhage)
- May present with symptoms of associated conditions (e.g. Sjogren’s syndrome - dry eyes and mouth-, arthritis, Raynaud’s phenomenon)
6
Q
Signs
A
Early - may be no signs
Late:
- Jaundice
- Skin pigmentation
- Scratch marks
- Xanthomas (secondary to hypercholesterolaemia)
- Hepatomegaly
- Ascites
- Signs of chronic liver disease
7
Q
Investigations
A
Bloods
LFT:
- 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
- Clotting: prolongation of PT
Typical features of PBC:
- Antimitochondrial antibodies (typical feature of PBC)
- High IgM
- High Cholesterol
- TFTs - because PBC is associated with autoimmune thyroid disease
Ultrasound
- Exclude extrahepatic biliary obstruction (e.g. gallstones)
Liver Biopsy
- In PBC, it will show chronic inflammatory cells and granulomas around the intrahepatic bile ducts, destruction of bile ducts, fibrosis and regenerating nodules of hepatocytes
