Liver disease of less common aetiology - primary biliary cirrhosis (PBC)
Description
- Renamed primary biliary cholangitis (not cirrhosis) - most patients never become cirrhotic
- Chronic, autoimmune, non-suppurative destructive cholangitis of small intralobular and septal bile ducts
- -> ductopenia -> cholestasis -> retained toxic bile acids -> fibrosis -> biliary cirrhosis
- Cholestatic biochemistry: ALP and GGT dominate; transaminases modestly raised
- A hepatitic pattern suggests AIH overlap
Histological stages (Ludwig)
| I | Florid duct lesion - granulomatous destruction of the septal duct |
| II | Ductular proliferation |
| III | Fibrosis / septal scarring |
| IV | Cirrhosis |
- Sampling variability is high - biopsy is not needed for diagnosis in a typical case
6 more sections, plus exam facts
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