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Primary Biliary Cirrhosis
(PBC, chronic non-suppurative destructive cholangitis)
- chronic disorder leading to destruction of bile ducts and cirrhosis
Epidemiology
- 90% in women age 40-50
- 7.5 / 100,00
Aetiology
- unknown, thought to be autoimmune
Presentation
Associated with other auto-immune conditions
Differential Diagnosis
- extrahepatic biliary obstruction
- autoimmune cholangitis
Investigations
- serum mitochondrial antibodies (AMA)
- >1:160 in 95%
- M2 specific to PBC
- ANA / Smooth
muscle antibodies may be present
- alkaline phosphatase
- raised
- may be only LFT abnormality
- serum cholesterol ↑
- Serum IgM may be very high
- USS
- diffuse alteration in liver structure
- Liver biopsy
- characteristically portal tract infiltrate of lymphocytes & plasma
cells (zone 1)
- loss of small bile ducts
- portal tract fibrosis
- cirrhosis
- hepatic granulomas
Treatment
Complications
Prognosis
- asymptomatic / pruritus may survive more than 20 years
- jaundice usually die within 5 years
- liver failure
- bleeding varices
- after transplantation 5year survival >70%
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