Primary biliary cholangitis: a comprehensive overview

Ana Lleo, Simona Marzorati, Juan Manuel Anaya, M. Eric Gershwin

Research output: Contribution to journalReview article

46 Scopus citations

Abstract

Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by biliary destruction, progressive cholestasis, and potentially liver cirrhosis. Patients develop a well-orchestrated immune reaction, both innate and adaptive, against mitochondrial antigens that specifically targets intrahepatic biliary cells. A puzzling feature of PBC is that the immune attack is predominantly organ specific, although the mitochondrial autoantigens are found in all nucleated cells. The disease results from a combination of genetic and environmental risk factors; however, the exact pathogenesis remains unclear. Serologically, PBC is characterized by presence of antimitochondrial antibodies, which are present in 90–95 % of patients and are often detectable years before clinical signs appear. Like other complex disorders, PBC is heterogeneous in its presentation, symptomatology, disease progression, and response to therapy. A significant number of patients develop end-stage liver disease and eventually require liver transplantation. Recent studies from large international cohorts have better identified prognostic factors, suggesting a change in patient management based on risk stratification. Therapeutic options are changing. In this review we discuss data on the autoimmune responses and treatment of the disease.

Original languageEnglish (US)
Pages (from-to)485-499
Number of pages15
JournalHepatology International
Volume11
Issue number6
DOIs
StatePublished - Nov 1 2017

Keywords

  • Antimitochondrial antibodies
  • Biliary epithelial cells
  • Epigenetics
  • Genetics
  • Obeticholic acid
  • Primary biliary cholangitis
  • Prognostic factors
  • UDCA

ASJC Scopus subject areas

  • Hepatology

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