Whether primary biliary cirrhosis recurs after orthotopic liver transp
lantation remains a controversial issue. Sixty consecutive patients wi
th primary biliary cirrhosis with at least 1 yr of follow-up after liv
er transplantation were studied. All patients were treated with triple
-drug immunosuppression (cyclosporine, prednisone, azathioprine). Hepa
tic biochemical parameters and protocol liver biopsy specimens were ev
aluated 1 wk, 3 wk, 4 mo and yearly after orthotopic liver transplanta
tion and at times of liver dysfunction. Antimitochondrial antibody tit
ers and IgM levels were determined at 4 mo and yearly. At the time of
last follow-up, all patients had marked symptomatic improvement compar
ed with their pretransplant condition, and 91% of the patients had nor
mal hepatic biochemical parameters, including serum levels of alkaline
phosphatase, bilirubin, ALT and IgM. In addition, all patients had si
gnificant decreases in antimitochondrial antibody titer (p = 0.0001) a
nd significant decreases in serum levels of IgM (p = 0.0001). Forty-on
e of the 60 patients had near-normal liver histological appearance. Of
those with abnormal histological appearance, five patients, 2 to 6 yr
after orthotopic liver transplantation, had histological features typ
ical of a florid duct lesion, suggesting recurrent primary biliary cir
rhosis. All five patients with portal granulomas had normal hepatic bi
ochemical values and were clinically asymptomatic. Two of the five pat
ients had persistent antimitochondrial antibody titers. We consider th
e documented histological changes highly suggestive of recurrence of p
rimary biliary cirrhosis after liver transplantation but, so far, have
no evidence that the condition in these patients is progressiVe.