152 - Primary biliary cirrhosis: clinical and diagnostic features
Autor(s): N. Bizzaro
Issue: RIMeL - IJLaM, Vol. 4, N. 2, 2008 (MAF Servizi srl ed.)
Page(s): 152-157
Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease of unknown etiology for which an autoimmune pathogenesis is supported by clinical and experimental data, including the presence of autoantibodies and autoreactive T cells. In fact, PBC diagnosis is based mainly on the detection of high titer serum anti-mitochondrial antibodies or PBC-specific antinuclear antibodies (nuclear lamin and multiple nuclear dots) which can precede the clinical symptoms of the disease by years. Several animal models have been described, while multiple candidates have been proposed to initiate disease in a genetically susceptible host by molecular mimicry. The natural history of the disease may vary widely, ranging from cases with a slow progression of disease over decades to patients having a rapid and fatal course. The only established medical treatment is based on ursodeoxycholic acid while liver transplantation is required in end stages