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Primary biliary cholangitis (PBC)

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an autoimmune liver disease characterized by immune-mediated biliary epithelial cell damage, cholestasis, and progressive fibrosis, characterized by pruritus, fatigue, and a growing awareness of mild cognitive impairment that eventually develops into biliary cirrhosis. In recent years, the incidence of autoimmune liver disease has increased significantly. Although up to 50% of patients are asymptomatic at diagnosis, present symptoms often has a significant impact on quality of life and functional status. Therefore, early detection, early detection and early treatment are especially important.

Specification


Clinical Significance

Primary biliary cholangitis (PBC) is an autoimmune liver disease with a high incidence in middle-aged women [1]. PBC specific antibodies mainly include anti-mitochondrial antibody (AMA), anti-nuclear antibody (ANA)-related nuclear membrane and polynuclear site antibodies. The main ANA antibody for PBC is anti-gp210 and anti-sp100, which is also included in the 2017 European Association of Liver Diseases diagnostic Guidelines for PBC [2]. The positive rate of anti-gp210 and anti-sp100 is 10% to 30% in PBC and they are highly specific for PBC [3].


References

[1] Lindor K D, Bowlus C L, Boyer J, et al. Primary biliary cholangitis: 2018 practice guidance from the American Association for the Study of Liver Diseases[J]. Hepatology, 2019, 69(1): 394-419.
[2] Sebode M, Weiler-Normann C, Liwinski T, et al. Autoantibodies in autoimmune liver disease—clinical and diagnostic relevance[J]. Frontiers in immunology, 2018, 9: 609.
[3] Granito A, Muratori P, Quarneti C, et al. Antinuclear antibodies as ancillary markers in primary biliary cirrhosis[J]. Expert review of molecular diagnostics, 2012, 12(1): 65-74.

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