Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
, 375 (12), 1161-70

Primary Sclerosing Cholangitis

Affiliations
Review

Primary Sclerosing Cholangitis

Konstantinos N Lazaridis et al. N Engl J Med.

Conflict of interest statement

No other potential conflict of interest relevant to this article was reported.

Disclosure forms provided by the authors are available with the full text of this article at NEJM.org.

Figures

Figure 1
Figure 1. Pathogenesis, Clinical Subtypes, and Outcomes of Primary Sclerosing Cholangitis
Primary sclerosing cholangitis is caused by the interaction of predisposing genetic factors and environmental exposures in local biologic processes that occur at the level of the gut (i.e., gut lymphocyte homing to liver and chronic mucosal inflammation) and the liver (i.e., activation of cholangiocytes and the bile milieu). This condition is influenced by the intestinal microbiome and other dynamic elements, including the epigenome and metabolome. The stochastic effect of environmental factors on germline and somatic biologic events leads to a variety of clinical subtypes of primary sclerosing cholangitis. The persistence and progressive nature of biologic dysfunction result in multifaceted, often interrelated, outcomes of primary sclerosing cholangitis. These outcomes range from chronic liver cholestasis and inflammation to malignant transformation of the liver and gut and to metabolic bone disease, and they may necessitate liver transplantation.

Comment in

Similar articles

See all similar articles

Cited by 48 PubMed Central articles

See all "Cited by" articles

MeSH terms

Substances

Feedback