Primary biliary cirrhosis

Question: A middle aged female is sent to your office with a normal LFT profile but a positive anti-mitochondrial antibody (AMA) on two consecutive blood draws.  She wants to know if she has primary biliary cirrhosis (PBC) and if she should be treated.  What should you advise?

A. no treatment; likely just false positive antibodies

B. no treatment; PBC may be present, but LFT is normal so the liver is probably normal

C. treat her PBC with ursodiol

D. not enough information make make treatment decisions

Answer: Primary biliary cirrhosis is a non-suppurative cholangitic condition of the intrahepatic biliary tree.  It occurs almost exclusively in women over age 30.  It should be suspected when presented with a cholestatic liver profile.  It may present with fatigue, pruritus, skin hyperpigmentation or musculoskeletal complaints.  Dyslipidemia and bone mineral abnormalities are common.

The AMA is 98% specific for PBC.  Either the patient has PBC, or at the very least the positive AMA is a harbinger for future disease.  A liver biopsy in this case will likely be normal or have early, non-diagnostic findings suggestive of PBC.

The risk of not treating includes progression of fibrosis if PBC is present; remember, it can lead to end-stage liver disease.  (There is no great evidence that initiation of ursodiol can prevent the onset of disease if it has yet to manifest itself).  Given the strong safety profile of ursodiol, the risk of treating is slim.  So…for the adamant patient, waiting for a rise in LFT to investigate/treat can be considered; otherwise, it is ok to begin treatment at this time.

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