Primary biliary cirrhosis is an autoimmune cholestatic liver disease which is diagnosed when 2 of the following 3 criteria are met:
- At least 6 months biochemical evidence of cholestatic liver disease
- Anti-mitochondrial antibody (AMA) positive
- Characteristic histological features on liver biopsy
- destruction of small and medium sized bile ducts
- non-supprative cholangitis
Females > males, 10:1
Onset usually around 5th decade of life
Around 2/3 of cases of primary biliary cirrhosis are diagnosed on the basis of blood tests, with the patient being otherwise asymptomatic. Other presentations are of pruritis (classically worse at night) and fatigue.
Around 2/3 of cases of primary biliary cirrhosis are diagnosed on the basis of blood tests, with the patient being otherwise asymptomatic. Other presentations are of pruritis (classically worse at night) and fatigue.
Blood tests
- LFTs show
- Raised ALP and gammaGT
- ALT, AST and bilirubin may be raised
- Raised PT only in advanced disease
- AMA – 90% sensitive, 95% specific
- ANA – non-specific, present in 30%
Complications
- Cirrhosis
- If cirrhosis present increased risk of hepatocellular carcinoma
- Portal hypertension
- Osteoporosis (1/3)
Treatment
- ursodeoxycholic acid
- liver transplant
- osteoporosis prophylaxis
References