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Primary Sclerosing Cholangitis
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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 Test
s 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
Cyst
ic 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
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