Antimitochondrial antibody is present in 95% patients with Primary Billiary Cirrhosis. The antibody assay is 95% sensitive and 98% specific for PBC (except if it's done by indirect immunoflorescence).
There has been suggestion that the presence of antimitochondrial antibodies may predict the eventual development of PBC in asymptomatic people based on a small study. About 13% of first-degree relatives of patients with PBC have circulating antimitochondrial antibodies, suggesting they may be susceptible to developing PBC. The clinical significance of this finding remains to be determined.
Here is a great review on Primary Billiary Cirrhosis.
*Immunofluorescent stain shows antimitochondrial antibodies on a liver biopsy specimen.
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