Primary Sclerosing Cholangitis (PSC)

Etiology

  • Autoimmune disorder causing cholestasis, intra and extra hepatic biliary duct inflammation and stenosis

Risk Factors

  • M > F
  • 70-90% have IBD (typically ulcerative colitis)
  • Avg age onset 40s

Symptoms

  • 50% identified when no sx, on basis of lab abnormalities
  • Progresses over 10-20 y to cirrhosis and end stage liver disease
  • Increased risk for cholangiocarcinoma, gall bladder cancer, and colon cancer (risk higher than UC alone)
  • Acute fever and pain if infection develops proximal to a stricture (e.g. post ERCP)

Physical Exam Findings

  • Sometimes hepatomegaly, splenomegaly, jaundice, ascites (later findings)

Tests