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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
(occurs in 2.5 to 7.5% of UC cases, and 60% to 70% co-
Incidence
)
Crohn's Disease
(rarely associated)
AIDS
Retroperitoneal fibrosis
Findings
Symptoms and Signs
Pruritus
Right Upper Quadrant Abdominal Pain
Jaundice
Fever
Weight loss
Malaise
Night Sweats
Labs
Liver Function Test
s suggestive of cholestasis
Bilirubin
elevated
Alkaline Phosphatase
elevated
Consider in any inflammatory bowel patient (esp.
Ulcerative Colitis
) with elevated
Alkaline Phosphatase
Diagnosis
ERCP
Stenosis and dilatation of biliary tract
Intrahepatic and extrahepatic bile ducts affected
Differential Diagnosis
Cholangiocarcinoma
Cholelithiasis
or
Cholecystitis
(also associated with
Inflammatory Bowel Disease
)
Caroli's Disease
Cyst
ic dilatation of bile ducts
Fasciola hepatica
Echinococcosis
Ascariasis
Course
Chronic, slowly progressive
Cholangitis
Progresses to liver failure,
Cirrhosis
with
Portal Hypertension
Mean survival: 12 years from diagnosis
Management
Pruritus
Cholestyramine
Bone preservation
Vitamin D Supplement
ation
Calcium Supplementation
Biliary obstruction treatment
Surgical drainage
End stage
Cirrhosis
Liver Transplant
ation
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