Primary Biliary Cirrhosis

By Disease Name > Primary Biliary Cirrhosis

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•	90% of patients = women; age 40-60

•	first symptom = pruritis; later jaundice and xanthomas (10%)

•	pathogenesis: probably autoimmune destruction of small intrahepatic biliary ducts

•	associated with Sjogren’s syndrome (75%) and CREST (3%)

•	diagnosis: early lack of symptoms but increased alkaline phosphatase (marked: 4-6X normal)

•	increased serum cholesterol

•	increased direct bilirubin

•	antimitochondrial antibody (90%)

•	definitive treatment = liver transplant