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Abstract

Primary sclerosing cholangitis is a chronic and intractably progressive cholangiopathy that leads to the development of fibrotic biliary strictures, cholangitis, biliary cirrhosis and end-stage liver disease. It portends a significant risk of hepatobiliary and colorectal malignancy. The disease is intimately linked with inflammatory bowel disease, and is often regarded as the classic hepatobiliary manifestation of colitis. There are currently no disease-modifying treatments that abrogate the progression to end-stage liver disease, though advances in the understanding of the underlying mechanisms of disease carry hope for improved risk stratification and treatment in the future.

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Correspondence to Gideon M. Hirschfield Ph.D., F.R.C.P. .

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Haldar, D., Hirschfield, G.M. (2015). Primary Sclerosing Cholangitis. In: Dixon, E., Vollmer Jr., C., May, G. (eds) Management of Benign Biliary Stenosis and Injury. Springer, Cham. https://doi.org/10.1007/978-3-319-22273-8_2

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  • DOI: https://doi.org/10.1007/978-3-319-22273-8_2

  • Publisher Name: Springer, Cham

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