Future therapies for primary sclerosing cholangitis

Craig Lammert, Raj Vuppalanchi

Research output: Chapter in Book/Report/Conference proceedingChapter


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.

Original languageEnglish (US)
Title of host publicationPrimary Sclerosing Cholangitis
Subtitle of host publicationCurrent Understanding, Management, and Future Developments
PublisherSpringer International Publishing
Number of pages14
ISBN (Electronic)9783319409085
ISBN (Print)9783319409061
StatePublished - Jan 1 2016


  • Apical sodium-dependent bile acid transport inhibitors
  • Bicarbonate umbrella
  • Farnesoid X receptor
  • Fibroblast growth factor 19
  • Gut adhesion molecules
  • Gut-liver Axis
  • Lysyl oxidase-like
  • Toxic bile acids

ASJC Scopus subject areas

  • Medicine(all)

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  • Cite this

    Lammert, C., & Vuppalanchi, R. (2016). Future therapies for primary sclerosing cholangitis. In Primary Sclerosing Cholangitis: Current Understanding, Management, and Future Developments (pp. 153-166). Springer International Publishing. https://doi.org/10.1007/978-3-319-40908-5_12