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primary sclerosing cholangitis

Saturday 17 September 2022




- neonatal sclerosing cholangitis
- auto-immune sclerosing cholangitis

Microscopy synopsis

- "onion skin" fibrosis around affected medium sized bile ducts;

  • smaller ducts rarely affected (except in small duct primary sclerosing cholangitis), meaning biopsy sampling rarely demonstrates this finding

- progressive atrophy of bile duct epithelium and obliteration of the lumen, leaving a fibrous scar
- ductular reaction and cholestasis may occur
- portal inflammatory infiltrate is typically mild and limited to biliary epithelium and portal tracts, though largest ducts may show increased infiltrate
- extravasated bile

  • may elicit xanthomatous or granulomatous response

- portal edema and portal fibrosis may be seen secondary to obstruction of larger ducts

Primary sclerosing cholangitis cirrhosis shows the typical biliary jigsaw pattern

Cholangiocarcinoma may occur in primary sclerosing cholangitis, preceded by intestinal metaplasia and biliary intraepithelial neoplasia (Am J Surg Pathol 2010;34:27)

Several staging systems exist, with the Ludwig system the most commonly used (Hepatology 2017;65:907):
- cholangitis / portal hepatitis
- periportal fibrosis or periportal hepatitis
- bridging fibrosis or bridging necrosis
- cirrhosis