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23 Cards in this Set
- Front
- Back
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mechanism of primary biliary cirrhosis
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Autoimmune, unknown etiology
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Pathogenesis of primary biliary cirrhosis
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destruction of small intrahepatic bile ducts->cholestasis
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Epidemiology of PBC
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95% women, 40-60
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Symptoms of PBC
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majority asymptomatic
also, fatigue and pruritis |
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Dx of PBC
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Lab studies->AP, AMA (90% +), inc serum IgM
Liver biopsy->granuloma, florid bile duct lesion Eval biliary tree for obstruction (won't see beads on string) |
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Complications of PBC
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dec bile acids in small bowel
-Ca and Vit D deficiency portal HTN |
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Management of PBC
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ursodiol -thins out bile
Vit and Ca cholestyramine for itching steroids NOT EFFECTIVE |
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Mechanism of Primary sclerosing cholangitis
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inflammatory, probably autoimmune
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Pathogenesis of PSC
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inflammation/sclerosis->
intra and/or extrahepatic duct-> cholestasis |
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Epidemiology of PSC
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predominantly males
75% of those w/ PSC have UC |
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Presentation of PSC
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asymptomatic (15-40%)
fatigue pruritus -also ab pain, jaundice, fever, HSM |
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Lab studies in PSC
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elevated serum AP
p-ANCA Hypergammaglobulinemia |
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Key imaging study for PSC
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cholangiogram (MRCP or ERCP)
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Liver bx findings on PSC
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onion skinning bile ducts
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PSC findings on ERCP
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beads on a string
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Complications of PSC
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pHTN, progressive liver dz
recurrent bacteremia cholelithiasis, choledocholithiasis cholangiocarcinoma (10-15% lifetime) Colon cancer |
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Management of PSC
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medical tx not effective
antibiotics endoscopic dilation liver transplant |
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Pathogenesis of autoimmune hepatitis
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hepatocellular injury
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general presentation of autoimmune hepatitis
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non-specific, general autoimmune disease in family
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Dx for autoimmune hepatitis
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female predominance (75-80%)
elevated AST and ALT elevated ANA >1:80 must have liver bx |
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liver bx findings in autoimmune hepatitis
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interface hepatitis
plasma cells |
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Most common autoimmune hepatitis findings on autoimmune screenings
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ANA and ASMA +
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Management of autoimmune hepatitis
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immunosuppression
-prednisone, azathioprine 2nd line for refractory dz -cyclosporine -tacrolimus -mycophenolate mofetil Lover transplant |
