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Primary sclerosing cholangitis: overview and update

Nature Reviews Gastroenterology & Hepatology volume 7, pages 611–619 (2010)Cite this article

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Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disorder that most commonly affects middle-aged men. PSC is strongly associated with IBD, and in this setting the risk of colorectal cancer is markedly increased. Cholangiocarcinoma, and its devastating consequences, is another well-recognized complication of PSC. This condition tends to progress to end-stage liver disease, and patients with PSC have reduced survival rates compared with the general population. Despite significant research efforts in this field, the pathogenetic mechanisms of PSC are still incompletely understood, although growing evidence supports the role of genetic and immunologic factors. Effective medical therapy is lacking; liver transplantation is the only curative treatment modality, with excellent outcomes in this patient population.

Key Points

  • Primary sclerosing cholangitis (PSC) is a progressive inflammatory and fibrosing condition, which can lead to chronic cholestasis and biliary cirrhosis

  • PSC has a strong association with IBD, with an increased risk of colorectal cancer in this setting

  • Patients with PSC have an increased risk of developing cholangiocarcinoma

  • The pathogenesis of PSC is not well defined but evidence points towards genetic and immunologic factors

  • Medical, endoscopic and surgical therapies that impact disease progression are currently not available; liver transplantation is the only therapeutic option, with excellent outcomes

  • Mortality rates from PSC have remained essentially unchanged over the past two decades

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Figure 1: Typical cholangiographic findings of primary sclerosing cholangitis.
Figure 2: Classic 'onion skin' fibrosis, causing obliteration of the bile ducts in primary sclerosing cholangitis.

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Authors and Affiliations

  1. Department of Medicine, University of Miami Miller School of Medicine, Miami VA Medical Center, 1201 NW 16th Street, Miami, 33125, FL, USA

    Flavia Mendes

  2. Department of Medicine, Mayo Clinic College of Medicine, 200 First Street, SW Rochester, 55905, MN, USA

    Keith D. Lindor

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F. Mendes and K. D. Lindor contributed equally to the research, discussion, writing and editing of the article.

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Correspondence to Keith D. Lindor.

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Mendes, F., Lindor, K. Primary sclerosing cholangitis: overview and update. Nat Rev Gastroenterol Hepatol 7, 611–619 (2010). https://doi.org/10.1038/nrgastro.2010.155

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