Pathophysiology
1) progressive inflammation and destruction of the intrahepatic bile ducts 2) causes development of cirrhosis 3) likely has an autoimmune component
Signs and Symptoms
1) fatigue 2) jaundice 3) pruritus 4) hepatomegaly 5) splenomegaly 6) xanthelasma 7) diagnosis is often made on asymptomatic laboratory detection
Characteristic Test Findings
Laboratory – 1) increased alkaline phosphatase (3 times normal value) 2) mildly elevated AST and ALT 3) antimitochondrial antibody
Histology/Gross Pathology
Ludwig’s classification describes histology – portal and periportal scarring and fibrosis and cirrhosis
Associated Conditions
1) osteoporosis 2) hypercholesterolemia 3) thyroid disease (15%) 4) scleroderma (15%) 5) joint pain 6) dry eyes/mouth (75%) 7) gallstones (30%)
Biochemistry
1) aberrant expression of class 2 HLA on bile duct surfaces 2) abnormalities in both cellular and humoral immune system causes T cell migration to bile ducts 3) autoantibodies are directed against protein on inner mitochondrial surface
Inheritance/Epidemiology
1) primarily affects middle-aged women 2) affects 40-150/million in general population
Treatment
1) ursodeoxycholic acid chronically 2) liver transplantation
Tips for USMLE
1) does not have increased risk of hepatoma 2) if question mentions anti-mitochondrial antibody, think promary biliary cirrhosis
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