Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
, 39 (4 Suppl 2), S90-S102

Pathophysiology of Cholangiopathies


Pathophysiology of Cholangiopathies

Mario Strazzabosco et al. J Clin Gastroenterol.


The diseases of the intrahepatic biliary tree are a large group of potentially evolutive congenital and acquired liver disorders affecting both the adult and pediatric populations. They represent a relevant cause of liver-related morbidity and mortality and an important indication for liver transplantation, particularly in children. While the practical approach to patients affected by biliary tree diseases has not significantly changed yet, the conceptual approach to the pathophysiology of cholangiopathies has witnessed important advances that will be discussed. The primary cell target of the pathogenetic sequence of these disorders is the biliary epithelium. Cholangiocytes have multifaceted functions, not limited to bile production. Their capability to secrete a range of different pro-inflammatory mediators, cytokines, and chemokines indicates a major role of cholangiocytes in the inflammatory reaction. Furthermore, paracrine secretion of growth factors and peptides mediates an extensive cross-talk with other liver cell types, including hepatocytes, stellate, and endothelial and inflammatory cells. Cholangiopathies share a number of pathogenetic mechanisms, including inflammation, cholestasis, fibrosis, apoptosis, altered development, and neoplastic transformation. These basic disease mechanisms will be discussed in detail, along with the distinct features of a number of cholangiopathies. Furthermore, an increase in the biliary cell compartment is a common response to many forms of liver injury, from cholangiopathies to viral and fulminant hepatitis. Elucidation of these pathophysiologic mechanisms will likely provide clues for future therapeutic strategies. Furthermore, understanding the role of cholangiocytes in liver regeneration/repair and the mechanisms of cholangiocyte activation and their relationship with liver progenitor cell will be of further interest.

Similar articles

  • Pathophysiology of the Intrahepatic Biliary Epithelium
    M Strazzabosco et al. J Gastroenterol Hepatol 15 (3), 244-53. PMID 10764023. - Review
    The intrahepatic bile duct epithelium modulates the fluidity and alkalinity of the primary hepatocellular bile from which it reabsorbs fluids, amino acids, glucose and bi …
  • The Intrahepatic Cholangiopathies
    A Birnbaum et al. Semin Liver Dis 18 (3), 263-9. PMID 9773426. - Review
    The intrahepatic biliary epithelial cells or cholangiocytes are the primary focus of injury in many congenital and acquired liver diseases of childhood. Although cholangi …
  • Heterogeneity of the Intrahepatic Biliary Epithelium
    S Glaser et al. World J Gastroenterol 12 (22), 3523-36. PMID 16773709. - Review
    The objectives of this review are to outline the recent findings related to the morphological heterogeneity of the biliary epithelium and the heterogeneous pathophysiolog …
  • Pathology and Pathogenesis of Intrahepatic Bile Duct Loss
    Y Nakanuma et al. J Hepatobiliary Pancreat Surg 8 (4), 303-15. PMID 11521175. - Review
    In recent years, the pathology and pathogenesis of bile duct loss have been extensively studied, and a number of hepatobiliary diseases have been added to the list of duc …
  • Clinical Implications of Novel Aspects of Biliary Pathophysiology
    M Marzioni et al. Dig Liver Dis 42 (4), 238-44. PMID 20167547. - Review
    Cholangiocytes are the epithelial cells that line the biliary tree; they are the target of chronic diseases termed cholangiopathies, which represent a daily challenge for …
See all similar articles

Cited by 60 PubMed Central articles

See all "Cited by" articles

Publication types

MeSH terms

LinkOut - more resources