Anti-gp210-positive primary biliary cholangitis: The dilemma of clinical treatment and emerging mechanisms

Ann Hepatol. 2023 Sep-Oct;28(5):101121. doi: 10.1016/j.aohep.2023.101121. Epub 2023 Jun 10.

Abstract

Anti-gp210 is the disease-specific anti-nuclear antibody (ANA) of primary biliary cholangitis (PBC). Anti-gp210-positive PBC patients have worse responses to ursodeoxycholic acid (UDCA) as compared with anti-gp210-negative patients. Moreover, anti-gp210-positive patients always present with more severe histopathologic features including lobular inflammation, interfacial hepatitis, and bile duct injury, and have a worse prognosis than their anti-gp210-negative counterparts. Previous studies have identified two antigenic epitopes recognized by anti-gp210. Although the pathogenetic mechanism of anti-gp210 production remains unclear, evidence suggests that the autoimmune response to anti-gp210 production might be due to molecular mimicry induced by bacteria or endogenous peptides. T cells and related cytokines play a critical role in the pathogenesis of PBC, however, the mechanism hasn't been fully understood. Thus, this review focuses on the clinicopathological characteristics of anti-gp210-positive PBC patients, the fundamental research of gp210 antigen, and the possible mechanism of anti-gp210 production to clarify the mechanism of anti-gp210-positive PBC and provide potential molecular targets for disease prevention and treatment in the future.

Keywords: Anti-gp210; Pathogenesis; Primary biliary cholangitis; Treatment response.

Publication types

  • Review

MeSH terms

  • Antibodies, Antinuclear
  • Autoantibodies
  • Cholangitis* / diagnosis
  • Cholangitis* / drug therapy
  • Humans
  • Liver Cirrhosis, Biliary* / diagnosis
  • Liver Cirrhosis, Biliary* / drug therapy
  • Nuclear Pore Complex Proteins
  • Prognosis
  • Ursodeoxycholic Acid / therapeutic use

Substances

  • Nuclear Pore Complex Proteins
  • Ursodeoxycholic Acid
  • Antibodies, Antinuclear
  • Autoantibodies