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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 (60% to 70% co-Incidence)
    2. Crohn's Disease (rarely)
  2. AIDS
  3. Retroperitoneal fibrosis
  • Symptoms and Signs
  1. Pruritus
  2. Right Upper Quadrant Abdominal Pain
  3. Jaundice
  4. Fever
  5. Weight loss
  6. Malaise
  1. Bilirubin elevated
  2. Alkaline Phosphatase elevated
  1. Stenosis and dilatation of biliary tract
  2. Intrahepatic and extrahepatic bile ducts affected
  • Differential Diagnosis
  1. Cholangiocarcinoma
  2. Caroli's Disease
    1. Cystic dilatation of bile ducts
  3. Fasciola hepatica
  4. Echinococcosis
  5. Ascariasis
  • Course
  1. Chronic, slowly progressive
  2. Cholangitis
  3. May progress to Cirrhosis with Portal Hypertension
  • 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