ABSTRACT
Introduction...................................................................................................... 407 Epidemiology of Primary Biliary Cirrhosis..................................................... 408 Serological Features of Primary Biliary Cirrhosis........................................... 408 Immunobiology of the Intrahepatic Biliary Epithelium .................................. 411 Lipoic Acid and Primary Biliary Cirrhosis...................................................... 412 Antibodies to Lipoic Acid in the Absence of Protein Carrier......................... 414 Significance of Reactivity to Lipoic Acid ....................................................... 414 Acknowledgments............................................................................................ 417 References ........................................................................................................ 417
Primary biliary cirrhosis (PBC) [1] is a progressive autoimmune liver disease with a 9:1 female predominance. Its peak incidence occurs over age 50, and it is uncommon under 25 years of age. The disease is characterized by immunemediated destruction of intrahepatic small bile ducts, leading to decreased bile secretion and the retention of toxic substances within the liver, further contributing to the development of fibrosis, cirrhosis, and eventual liver failure. Understanding the etiology of PBC remains a challenge. However, recent studies have provided new insights into the events leading to the breakdown of self-tolerance and development of autoimmunity in PBC. In this article we will review emerging data and discuss the potential link between immune system dysregulation and the immunopathology of PBC.
