AMA are detected in the serum of 92% to 95% of patients with PBC using indirect immunofluorescent methods. AIC is the term used to describe the 5% to 8% of AMA-negative PBC patients who uniformly have ANA and SMA. Recent applications of more sensitive and specific tests to detect serum AMA have shown that most, if not all, patients with AIC actually do have AMA. Emerging evidence that AMA and mitochondrial autoantigens play important roles in the immunopathogenesis of NSDC also suggests that AIC and PBC are likely to be a single disease, exhibiting variation in the types of autoantibodies and in both the concentrations and immunoglobulin isotypes of AMA.