Pathology: florid duct lesion

Unlike primary sclerosing cholangitis (PSC), the diagnosis of primary biliary cirrhosis (PBC) is made histologically.  Liver biopsy should be pursued if a cholestatic LFT profile is noted in a female patient with a positive anti-mitochondrial antibody (AMA).

The florid duct lesion is used to describe the textbook finding of PBC.  In the portal triad, inflammatory cells will surround the bile duct and duct-infiltrating lymphocytes may be present (there are several present in this irregularly shaped bile duct).  In this specimen, there is also a periportal rim of cellular debris- granulomatous tissue.  Remember, PBC is in the differential diagnosis of hepatic granulomas. 

An AMA should be present in this patient; this serum marker has excellent sensitivity and specificity for the diagnosis.  Ursodeoxycholic acid (15 mg/kg) should be given as treatment.  It delays the progression to end-stage liver disease, enhances survival, and is well-tolerated.  If LFT have not returned to normal after 6-12 months of therapy, an alternative treatment should be attempted, such as colchicine or methotrexate.

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