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Future Therapies for Primary Sclerosing Cholangitis

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Primary Sclerosing Cholangitis

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Abstract

Primary sclerosing cholangitis (PSC) is a rare disease characterized by inflammation and fibrosis of the intra- and extrahepatic bile ducts leading to biliary strictures, parenchymal fibrosis, and subsequent progression to cirrhosis and liver failure. The disease is often associated with concomitant inflammatory bowel disease (IBD) and has varied phenotype and disease progression. PSC is an orphan disease, and currently there is no effective treatment other than liver transplantation. Fortunately, recent breakthroughs in the understanding of the pathogenesis of liver diseases have unraveled several new targets for treatment of PSC. In this chapter, we review the current understanding of the pathogenesis of PSC and the rationale behind the novel therapeutic interventions. The launch of additional trials will bring us a step closer to new treatments and an eventual cure for PSC.

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  1. Division of Gastroenterology and Hepatology, Indiana University School of Medicine, Indianapolis, IN, 46202, USA

    Craig Lammert MD & Raj Vuppalanchi MD

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  1. Craig Lammert MD
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Correspondence to Raj Vuppalanchi MD .

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  1. Department of Gastroenterology-Hepatology, University of Colorado, Aurora, Colorado, USA

    Lisa M. Forman

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Lammert, C., Vuppalanchi, R. (2017). Future Therapies for Primary Sclerosing Cholangitis. In: Forman, L. (eds) Primary Sclerosing Cholangitis. Springer, Cham. https://doi.org/10.1007/978-3-319-40908-5_12

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