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Primary Sclerosing Cholangitis
Aka: Primary Sclerosing Cholangitis
- Epidemiology
- Younger men most often affected
- Peak ages affected: 25-45 years old
- Pathophysiology
- Biliary tree sclerosing inflammation and fibrosis
- Affects intrahepatic and extrahepatic bile ducts
- Associated Conditions
- Inflammatory Bowel Disease
- Ulcerative Colitis (60% to 70% co-Incidence)
- Crohn's Disease (rarely)
- AIDS
- Retroperitoneal fibrosis
- Symptoms and Signs
- Pruritus
- Right Upper Quadrant Abdominal Pain
- Jaundice
- Fever
- Weight loss
- Malaise
- Labs: Liver Function Tests suggestive of cholestasis
- Bilirubin elevated
- Alkaline Phosphatase elevated
- Diagnosis: ERCP
- Stenosis and dilatation of biliary tract
- Intrahepatic and extrahepatic bile ducts affected
- Differential Diagnosis
- Cholangiocarcinoma
- Caroli's Disease
- Cystic dilatation of bile ducts
- Fasciola hepatica
- Echinococcosis
- Ascariasis
- Course
- Chronic, slowly progressive
- Cholangitis
- May progress to Cirrhosis with Portal Hypertension
- Management
- Pruritus
- Cholestyramine
- Bone preservation
- Vitamin D Supplementation
- Calcium Supplementation
- Biliary obstruction treatment
- Surgical drainage
- End stage Cirrhosis
- Liver transplantation
