II. Epidemiology
- Younger men most often affected
- Peak ages affected: 25-45 years old
III. Pathophysiology
- Biliary tree sclerosing inflammation and fibrosis
- Affects intrahepatic and extrahepatic bile ducts
IV. Associated Conditions
-
Inflammatory Bowel Disease
- Ulcerative Colitis (occurs in 2.5 to 7.5% of UC cases, and 60% to 70% co-Incidence)
- Crohn's Disease (rarely associated)
- AIDS
- Retroperitoneal fibrosis
V. Findings: Symptoms and Signs
- Pruritus
- Right Upper Quadrant Abdominal Pain
- Jaundice
- Fever
- Weight loss
- Malaise
- Night Sweats
VI. Labs: Liver Function Tests suggestive of cholestasis
- Bilirubin elevated
-
Alkaline Phosphatase elevated
- Consider in any inflammatory bowel patient (esp. Ulcerative Colitis) with elevated Alkaline Phosphatase
VII. Diagnosis: ERCP
- Stenosis and dilatation of biliary tract
- Intrahepatic and extrahepatic bile ducts affected
VIII. Differential Diagnosis
- Cholangiocarcinoma
- Cholelithiasis or Cholecystitis (also associated with Inflammatory Bowel Disease)
- Caroli's Disease
- Cystic dilatation of bile ducts
- Fasciola hepatica
- Echinococcosis
- Ascariasis
IX. Course
- Chronic, slowly progressive
- Cholangitis
- Progresses to liver failure, Cirrhosis with Portal Hypertension
- Mean survival: 12 years from diagnosis
X. Management
- Pruritus
- Bone preservation
- Biliary obstruction treatment
- Surgical drainage
- End stage Cirrhosis
- Liver Transplantation