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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. 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