Primary sclerosing cholangitis

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Background

  • 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

  • Generally asymptomatic but can present with fatigue, abdominal pain, jaundice, cholangitis, puritis, weight loss, or fever
  • Mean age at presentation: 30-40

Differential Diagnosis

Evaluation

  • 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

Management

  • 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)

Disposition

See Also

References

  • Current Clinical Medicine, 2nd edition by Cleveland Clinic