Antimitochondrial Antibody-Negative Primary Biliary Cirrhosis

Primary biliary cirrhosis (PBC) is considered a classic autoimmune disease insofar as 95% of patients are seropositive for specific antimitochondrial antibodies (AMA). Yet it is still the case that the remaining 5% of patients have equivalent disease but test persistently AMA negative. Although variations exist in the immunologic profile of these patients, clinically there are no discernable differences, and although the diagnostic threshold is higher, the treatment and prognosis mirror that of classic disease. Previous terminology, therefore, has been abandoned in favor of the description, AMA-negative PBC.