PowerPoint Presentation - Pathology of Liver, V by zdn12589

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									Histopathology of PBC
     Primary biliary cirrhosis: overview

o   Chronic disease showing non-suppurative
    slowly progressive destruction of intrahepatic
    bile ducts (40-80 micrometer)
o   Primarily middle-aged women (F:M = 9:1)
o   Autoimmune etiology: > 95% have circulating
    anti-mitochondrial antibodies
        PBC: clinical diagnosis & management

o   Insidious onset pruritus, +/- jaundice
o   Lab data and biopsy complementary:
    o   Elevated serum alkaline phosphatase & GGT
    o   Serum anti-mitochondrial antibodies present
    o   Biopsy: compatible with PBC? Degree of fibrosis?
        Is cirrhosis present?
Normal Liver Biopsy
PBC: early histopathology
NRH
            PBC: later histopathology
o   Progressive destruction/disappearance of bile ducts <
    70 microns diameter
o   Progressive appearance of fibrosis: portal, bridging,
    eventual cirrhosis. Strongest prognostic indicator is
    degree of fibrosis.
o   Problems for biopsy interpretation:
    o   After the early florid bile duct lesion with granulomatous
        inflammation, histologic changes resemble chronic viral
        hepatitis (need clinical & lab)
    o   As cirrhosis develops, distinctive features mostly obliterated,
        except for absent bile ducts <70 micron
    o   Uneven histopathologic changes
Micronodular cirrhosis
Macronodular cirrhosis
          Pathological Stages (4)
   1 Destruction of bile ducts in portal tracts
   2 Inflammation beyond portal tracts
   3 fibrous septa link portal triads
   Cirrhosis
         Liver transplantation
   Only effective Rx for liver failure
   Survival is excellent 85% at 5 years
   CAN RECUR IN GRAFT-30% AT 10 YEARS
     AIH-PBC Overlap Syndrome
   AIH                              PBC
       ALT 5X                           AMA +
       IgG 2X                           Florid bile duct lesion
       ASMA +                           Alk Ph. 5X
       Lymphocytic Interface
        Activity: mod to severe
The End

								
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