Clinical Reviews in Allergy & Immunology

, Volume 42, Issue 3, pp 342–354

Cutting-Edge Issues in Primary Biliary Cirrhosis

  • Marco Folci
  • Francesca Meda
  • M. Eric Gershwin
  • Carlo Selmi
Article

Abstract

Several crucial issues remain open in our understanding of primary biliary cirrhosis (PBC), an autoimmune liver disease targeting the small- and medium-sized intrahepatic bile ducts. These issues include the high tissue specificity of the autoimmune injury despite the nontraditional autoantigens found in all mitochondria recognized by PBC-associated autoantibodies, the causes of the commonly observed pruritus, and the disease etiology per se. In all these fields, there has been recent interest secondary to the use of large-scale efforts (such as genome-wide association studies) that were previously considered poorly feasible in a rare disease such as PBC as well as other intuitions. Accordingly, there are now fascinating theories to explain the onset and severity of pruritus due to elevated autotaxin levels, the peculiar apoptotic features of bile duct cells to explain the tissue specificity, and genomic and epigenetic associations contributing to disease susceptibility. We have arbitrarily chosen these four aspects as the most promising in the PBC recent literature and will provide herein a discussion of the recent data and their potential implications.

Keywords

Autoimmune cholangitis Autotaxin GWAS Epigenetics Apoptosis 

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Marco Folci
    • 1
  • Francesca Meda
    • 1
    • 2
  • M. Eric Gershwin
    • 3
  • Carlo Selmi
    • 1
    • 2
  1. 1.Division of Internal MedicineIRCCS Istituto Clinico HumanitasMilanItaly
  2. 2.Department of Translational MedicineUniversità degli Studi di MilanoMilanItaly
  3. 3.Division of Rheumatology, Allergy, and Clinical ImmunologyUniversity of CaliforniaDavisUSA

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