Antinuclear antibodies specific for primary biliary cirrhosis

The serological hallmark of primary biliary cirrhosis (PBC) is the presence of antimitochondrial antibodies. However, antinuclear antibodies (ANA) are also detectable in approximately 50% of subjects with PBC. Most clinical laboratories use indirect immunofluorescence microscopy to detect ANA and tow labeling patterns that predominate in PBC are punctate nuclear rim' and multiple nuclear dots. Work over the past several years has shown that antibodies giving these paterna most often recognize nuclear pore membrane protein gp210 and nuclear body protein sp100, respectively. These ANA are highly specific for PBC and detected in approximately 25% of patients. Less frequently, ANA apparently unique to PBC recognize other proteins of the nuclear envelope and nuclear bodies. While antibodies against gp210, sp100 and some other nuclear proteins are very specified to PBC and may therefore be useful diagnostic marker and their connection to pathogenesis remains to be elucidates. (C) 2003 Elsever Science B.V All rights reserved.