Primary sclerosing cholangitis (PSC)

• Disease of unknown origin characterised by inflammation, fibrosis and strictures in the intra and extra-hepatic bile ducts
• Possible autoimmune
• Chronic biliary obstruction and secondary cirrhosis leads to liver faiure
• Patients may be asymptomatic of may have puritis, jaundice, abdo pain or fatigue
• It is associated with UC – 3% of UC patients have PSC but 80% with PSC have UC/Crohn’s

Signs

• Jaundice
• Hepatomegaly
• Portal hypertension

Complications

• Bacterial cholangitis
• Cholangiocarcinoma
• Increased risk of colorectal cancer
• Some overlap with Type I autoimmune hepatitis

Tests

• Bloods
  • Increased alk phos followed by increased bilirubin
  • Hypergammaglobulinaemia
  • AMA neg but may be ANA, SMA, ANCA positive
• ERCP – characteristic beaded appearance
• Liver biopsy – fibrous obliterative cholangitis

Management

• Colestyramine for puritis
• Ursodeoxycholic acid
• Antibiotics for bacterial cholangitis
• Endoscopic stenting may help improve symptomatic strictures
• Yearly ultrasound screening for cholangiocarcinoma
• Liver transplantation
• Colonoscopy screening yearly