Nonspecific antinuclear antibodies (ANA) and/or smooth muscle antibodies are found in serum of one third of patients with otherwise clear-cut PBC [48] , but are of limited diagnostic value. In contrast, specific ANA directed against nuclear body or envelope proteins such as anti-Sp100, presenting as multiple (6) (7) (8) (9) (10) (11) (12) nuclear dots at indirect immunofluorescence staining and anti-gp210, presenting as perinuclear rims have shown a specificity of >95% for PBC, although their sensitivity is low. These specific ANA can be used as diagnostic markers for PBC in the absence of AMA titers [29] .