Antimitochondrial and antinuclear antibodies in primary biliary cirrhosis

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Antimitochondrial and antinuclear antibodies in primary biliary cirrhosis

Journal Title: Postępy Nauk Medycznych - Year 2011, Vol 24, Issue 11

Abstract

Primary biliary cirrhosis (PBC) is a chronic liver disease of unknown etiology. It starts with inflammatory changes in the small biliary ducts and slowly develops pathological changes in the tissue, leading to complete liver cirrhosis. Serum autoantibodies are crucial tools for diagnosis of PBC. Antimitochondrial autoantibodies (AMA), directed against the 2-oxoacid-dehydrogenase complex of the inner mitochondrial membrane, are the most sensitive and specific immunologic markers of PBC. Additionally 50% of PBC patients have antinuclear antibodies (ANA). The nuclear envelope proteins have been identified as target of ANA reactivity. A part of PBC patients develops antibodies against constituents of the nuclear pore complex – gp210 protein, p62 nucleoporin and lamin B receptor (LBR). Antibodies to these antigens such as gp210 protein and p62 nucleoporin are highly specific for PBC and can aid in the serologic diagnosis, especially in cases in which antimitochondrial antibodies are not detectable. A review of several studies performed in various regions of the world showed wide differences in prevalence (9-45%) of antibodies against gp210 protein in PBC. There are reports indicating an association of positivity for antibodies against the nuclear pore complex with poor outcome of PBC. During the last years a number of another nuclear structures have been recognized as a specific targets of ANAs in PBC. These include Sp100 and promyelocytic leukemia proteins. Specific antinuclear antibodies will provide an important, novel clinical tool in diagnostic process of patients with PBC.

Authors and Affiliations

Alicja Bauer

Keywords

primary biliary cirrhosis Autoantibodies Liver Autoimmunity nuclear pore complex pierwotna żółciowa marskość wątroba autoprzeciwciała

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