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Diagnosis and Treatment: ERCP in PSC

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Abstract

Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic disease characterized by inflammatory destruction and fibrosis of both intra- and extra-hepatic bile ducts. Seen primarily in patients with underlying inflammatory bowel disease who have an increased risk of colorectal cancer, the most feared biliary complication of PSC is cholangiocarcinoma. Endoscopic cholangiopancreatography (ERCP) is a very useful diagnostic and therapeutic tool in the management of PSC and cholangiocarcinoma.

In this chapter we review our current understanding of this complex biliary disorder, which includes the risk factors, pathogenesis, differential diagnosis, overlap syndromes, complications including cholangiocarcinoma, the role of ERCP in the diagnosis and management of complications related to PSC/cholangiocarcinoma, and the role of other noninvasive techniques such as magnetic resonance cholangiopancreatography (MRCP) and endoscopic ultrasound (EUS) in the diagnosis of PSC.

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318160_1_En_22_MOESM1_ESM.mp4

This 51-year-old patient has inactive chronic ulcerative colitis and progressive cholestasis from primary sclerosing cholangitis. Diagnostic ERCP demonstrates multiple intra- and extra-hepatic strictures and stones treated with a small biliary sphincterotomy, stricture dilation, stone extraction, brush cytology, and placement of short-term right and left hepatic duct stents. Bilirubin fell from 14 mg/dL to 1.0 and alkaline phosphatase levels decreased threefold for >1 year post-endoscopic treatment. (WMV 18638 kb)

Reticular pattern of biliary epithelium manifest by thick dark bands and clumps in a patient with cholangicarcinoma as visualized with confocal endomicroscopy (Cellvizio, Mauna Kea Technologies, Paris, France). Thick white bands are vessels. Image courtesy of Ian Gan MD, Virginia Mason Medical Center. (WMV 18638 kb)

Video 22.1

This 51-year-old patient has inactive chronic ulcerative colitis and progressive cholestasis from primary sclerosing cholangitis. Diagnostic ERCP demonstrates multiple intra- and extra-hepatic strictures and stones treated with a small biliary sphincterotomy, stricture dilation, stone extraction, brush cytology, and placement of short-term right and left hepatic duct stents. Bilirubin fell from 14 mg/dL to 1.0 and alkaline phosphatase levels decreased threefold for >1 year post-endoscopic treatment. (WMV 18638 kb)

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Srinivasan, N., Kozarek, R. (2015). Diagnosis and Treatment: ERCP in PSC. In: Kozarek, R., Chiorean, M., Wallace, M. (eds) Endoscopy in Inflammatory Bowel Disease. Springer, Cham. https://doi.org/10.1007/978-3-319-11077-6_22

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