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.