II. Epidemiology

  1. Younger men most often affected
  2. Peak ages affected: 25-45 years old

III. Pathophysiology

  1. Biliary tree sclerosing inflammation and fibrosis
  2. Affects intrahepatic and extrahepatic bile ducts

IV. Associated Conditions

  1. Inflammatory Bowel Disease
    1. Ulcerative Colitis (occurs in 2.5 to 7.5% of UC cases, and 60% to 70% co-Incidence)
    2. Crohn's Disease (rarely associated)
  2. AIDS
  3. Retroperitoneal fibrosis

V. Findings: Symptoms and Signs

VI. Labs: Liver Function Tests suggestive of cholestasis

  1. Bilirubin elevated
  2. Alkaline Phosphatase elevated
    1. Consider in any inflammatory bowel patient (esp. Ulcerative Colitis) with elevated Alkaline Phosphatase

VII. Diagnosis: ERCP

  1. Stenosis and dilatation of biliary tract
  2. Intrahepatic and extrahepatic bile ducts affected

VIII. Differential Diagnosis

  1. Cholangiocarcinoma
  2. Cholelithiasis or Cholecystitis (also associated with Inflammatory Bowel Disease)
  3. Caroli's Disease
    1. Cystic dilatation of bile ducts
  4. Fasciola hepatica
  5. Echinococcosis
  6. Ascariasis

IX. Course

  1. Chronic, slowly progressive
  2. Cholangitis
  3. Progresses to liver failure, Cirrhosis with Portal Hypertension
  4. Mean survival: 12 years from diagnosis

X. Management

  1. Pruritus
    1. Cholestyramine
  2. Bone preservation
    1. Vitamin D Supplementation
    2. Calcium Supplementation
  3. Biliary obstruction treatment
    1. Surgical drainage
  4. End stage Cirrhosis
    1. Liver Transplantation

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