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Review
. 2016 Sep 22;375(12):1161-70.
doi: 10.1056/NEJMra1506330.

Primary Sclerosing Cholangitis

Konstantinos N Lazaridis  1 , Nicholas F LaRusso  1
Affiliations
Review

Primary Sclerosing Cholangitis

Konstantinos N Lazaridis et al. N Engl J Med. .
No abstract available

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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.
See this image and copyright information in PMC

Comment in

  • Primary Sclerosing Cholangitis.
    Lazaridis KN, LaRusso NF. Lazaridis KN, et al. N Engl J Med. 2016 Dec 22;375(25):2501-2502. doi: 10.1056/NEJMc1613273. N Engl J Med. 2016. PMID: 28002707 No abstract available.
  • Primary Sclerosing Cholangitis.
    Noroski LM, Fuleihan R. Noroski LM, et al. N Engl J Med. 2016 Dec 22;375(25):2501. doi: 10.1056/NEJMc1613273. N Engl J Med. 2016. PMID: 28002708 No abstract available.
  • Primary Sclerosing Cholangitis.
    Valente R, Del Chiaro M, Arnelo U. Valente R, et al. N Engl J Med. 2016 Dec 22;375(25):2500-1. doi: 10.1056/NEJMc1613273. N Engl J Med. 2016. PMID: 28025879 No abstract available.

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References

    1. Eaton JE, Talwalkar JA, Lazaridis KN, Gores GJ, Lindor KD. Pathogenesis of primary sclerosing cholangitis and advances in diagnosis and management. Gastroenterology. 2013;145:521–36. - PMC - PubMed
    1. Hirschfield GM, Karlsen TH, Lindor KD, Adams DH. Primary sclerosing cholangitis. Lancet. 2013;382:1587–99. - PubMed
    1. Lazaridis KN, LaRusso NF. The cholangiopathies. Mayo Clin Proc. 2015;90:791–800. - PMC - PubMed
    1. Jussila A, Virta LJ, Pukkala E, Färkkilä MA. Malignancies in patients with inflammatory bowel disease: a nationwide register study in Finland. Scand J Gastroenterol. 2013;48:1405–13. - PubMed
    1. Molodecky NA, Kareemi H, Parab R, et al. Incidence of primary sclerosing cholangitis: a systematic review and metaanalysis. Hepatology. 2011;53:1590–9. - PubMed

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