AMA autoantibodies are pathognomonic for PBC and lead to the diagnosis with a high specificity and sensitivity. AMA-positive individuals, even if no signs of cholestasis and/or liver inflammation are present, are very likely to develop PBC. Mitchison et al. [36] , in a small study, evaluated liver pathology of 29 asymptomatic AMApositive (levels>1:40) individuals lacking AP elevation. At inclusion, all but two had abnormal liver histology, and in 12, findings were diagnostic for PBC. A 10-year followup of these subjects revealed that 24 of the 29 remained AMA-positive; all 24 developed biochemical evidence of cholestasis and 22 became symptomatic [37] , confirming a high positive predictive value of positive AMA testing for the development of PBC.