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Primary Sclerosing Cholangitis

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Primary Sclerosing Cholangitis

  • Epidemiology
  1. Younger men most often affected
  2. Peak ages affected: 25-45 years old
  • Pathophysiology
  1. Biliary tree sclerosing inflammation and fibrosis
  2. Affects intrahepatic and extrahepatic bile ducts
  • 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
  • Findings
  • Symptoms and Signs
  1. Pruritus
  2. Right Upper Quadrant Abdominal Pain
  3. Jaundice
  4. Fever
  5. Weight loss
  6. Malaise
  7. Night Sweats
  1. Bilirubin elevated
  2. Alkaline Phosphatase elevated
    1. Consider in any inflammatory bowel patient (esp. Ulcerative Colitis) with elevated Alkaline Phosphatase
  1. Stenosis and dilatation of biliary tract
  2. Intrahepatic and extrahepatic bile ducts affected
  • 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
  • Course
  1. Chronic, slowly progressive
  2. Cholangitis
  3. Progresses to liver failure, Cirrhosis with Portal Hypertension
  4. Mean survival: 12 years from diagnosis
  • 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