Primary sclerosing cholangitis

Gideon M. Hirschfield, Tom H. Karlsen, Keith Lindor, David H. Adams

Research output: Contribution to journalArticle

257 Citations (Scopus)

Abstract

Primary sclerosing cholangitis is the classic hepatobiliary manifestation of inflammatory bowel disease and is generally chronic and progressive. Patients frequently present with asymptomatic, anicteric cholestasis, but many develop progressive biliary strictures with time, leading to recurrent cholangitis, biliary cirrhosis, and end-stage liver disease. Medical treatment does not slow the progression of disease, and many patients need liver transplantation, after which recurrent disease is a risk. The increased incidence of hepatobiliary cancer, which is not related to the underlying severity of biliary fibrosis, is of particular concern. Risk of colorectal cancer is also increased in patients with coexistent inflammatory bowel disease. Mechanistic insights have arisen from studies of secondary sclerosing cholangitis, in which a similar clinical profile is associated with a specific cause, and genomic studies have elucidated potential disease-initiating pathways in the primary form. The close association between inflammatory bowel disease and primary sclerosing cholangitis underscores the need to further understand the role of environmental factors in generation of lymphocytes that are postulated to be retargeted, deleteriously, to the biliary tree. Treatment of primary sclerosing cholangitis is confined to supportive measures, but advances in pathobiology suggest that new stratified approaches will soon be available.

Original languageEnglish (US)
Pages (from-to)1587-1599
Number of pages13
JournalThe Lancet
Volume382
Issue number9904
DOIs
StatePublished - 2013

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Sclerosing Cholangitis
Inflammatory Bowel Diseases
End Stage Liver Disease
Cholangitis
Biliary Liver Cirrhosis
Cholestasis
Biliary Tract
Liver Transplantation
Disease Progression
Colorectal Neoplasms
Pathologic Constriction
Fibrosis
Lymphocytes
Incidence
Therapeutics
Neoplasms

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Hirschfield, G. M., Karlsen, T. H., Lindor, K., & Adams, D. H. (2013). Primary sclerosing cholangitis. The Lancet, 382(9904), 1587-1599. https://doi.org/10.1016/S0140-6736(13)60096-3

Primary sclerosing cholangitis. / Hirschfield, Gideon M.; Karlsen, Tom H.; Lindor, Keith; Adams, David H.

In: The Lancet, Vol. 382, No. 9904, 2013, p. 1587-1599.

Research output: Contribution to journalArticle

Hirschfield, GM, Karlsen, TH, Lindor, K & Adams, DH 2013, 'Primary sclerosing cholangitis', The Lancet, vol. 382, no. 9904, pp. 1587-1599. https://doi.org/10.1016/S0140-6736(13)60096-3
Hirschfield GM, Karlsen TH, Lindor K, Adams DH. Primary sclerosing cholangitis. The Lancet. 2013;382(9904):1587-1599. https://doi.org/10.1016/S0140-6736(13)60096-3
Hirschfield, Gideon M. ; Karlsen, Tom H. ; Lindor, Keith ; Adams, David H. / Primary sclerosing cholangitis. In: The Lancet. 2013 ; Vol. 382, No. 9904. pp. 1587-1599.
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