Primary sclerosing cholangitis


  • Autoimmune disease typically seen in young men
  • Progressive inflammation and fibrosis of intra/extra hepatic bile ducts
  • Most (80%) cases are associated with inflammatory bowel disease, typically ulcerative colitis, 10% of patients with ulcerative colitis have PSC
  • Increased risk of colon cancer in patients with Ulcerative colitis and PSC (more than UC alone) Increased risk of cholangiocarcinoma
  • Prevalence is 1 to 6 per 100,000 in the U.S

Clinical Features

Differential Diagnosis

  • Autoimmune hepatitis
  • Primary biliary cirrhosis
  • IgG4 related disease


  • LFTs
    • Alkaline phosphatase is usually elevated with mild elevations in aminotransferases
    • Bilirubin is typically normal, except when common hepatic duct or common bile duct is involved in late stages of disease
  • Perinuclear antineutrophil cytoplasmic antibody (pANCA) positive in 2/3rds of cases
  • Cholangiography
  • Diagnosis made by ERCP or MRCP, which demonstrates strictures or beading of the intrahepatic or extrahepatic bile ducts
  • Liver biopsy typically shows pericholangitis and periductual fibrosis but is often not diagnostic in early disease
  • Consider serum IgG4 levels


  • High dose Ursodeoxycholic acid (UDCA), 25-30mg/kg/day
    • May improve liver chemistries but does not slow disease progression and may actually hasten development of portal hypertension
  • Periodic dilation of strictures via ERCP or percutaneous route
  • Liver Transplant should be offered to those with advanced liver disease or repeated bouts of cholangitis (disease can recur after transplantation)


See Also


  • Current Clinical Medicine, 2nd edition by Cleveland Clinic