Incidence
Age	- mean age at presentation is 39 years
Sex	- M70% of patients are male
Geography
Aetiology	- inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts -> multiple areas of narrowing throughout biliary system - HLA associations - reduced incidence in smokers - 75% of patients have IBD - patients with AIDS can develop PSC - may be due to Cryptosporidium parvum infection
Presentation	- 60% are asymptomatic intially, diagnosed on raised ALP + -ve anti-mitochondrial antibodies (c.f. primary biliary cirrhosis) History - fluctuating pruritus, jaundice ± abdominal pain - may develop cirrhosis and portal hypertension
Investigations	Liver biopsy ERCP - multiple strictures
Macro
Micro	- fibrous obliterating cholangitis - onion-skin lesion with dense connective tissue surrounding a necrotic, often obliterated bile duct
Staging
Serum markers	- 80% have myeloperoxidase ANCA antibodies
Management	Medical - steroids, methotrexate and ursodeoxycholate seem to help some patients Surgical - OLT should be considered in patients with advanced disease - extrahepatic biliary strictures can be dilated or stented with some short-term improvement in symptoms and biochemistry
Prognosis	Symptomatic patients - mean survival time from diagnosis to death or transplantation is 12 years Asymptomatic patients - 75% are alive at 15 years Liver transplantation - 90% survival at 5 years
Complications	- HCC and cholangiocarcinoma

Hepatobiliary medicine

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