Management of Primary Sclerosing Cholangitis: Conventions and Controversies

Primary sclerosing cholangitis (PSC) is a chronic inflammatory cholangiopathy that results in fibrotic strictures and dilations of the intra- and extrahepatic bile ducts. PSC is uncommon, occurs predominantly in males and has a strong association with inflammatory bowel disease. While the pathogenesis of PSC has not been fully elucidated, emerging evidence supports roles for the innate and adaptive immune systems, and genome-wide analyses have identified several genetic associations. Using the best available evidence, the present review summarizes the current understanding of the diagnosis, pathogenesis and management of PSC. Despite its rarity, there is an urgent need for collaborative research efforts to advance therapeutic options for PSC beyond liver transplantation.

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Primary Sclerosing Cholangitis

10.2310/gastro.5472 ◽

2015 ◽

Author(s):

Udayakumar Navaneethan

Keyword(s):

Differential Diagnosis ◽

Primary Sclerosing Cholangitis ◽

Sclerosing Cholangitis ◽

Biliary Tree ◽

Biliary Cirrhosis ◽

Extrahepatic Bile Ducts ◽

Biliary Stasis ◽

Inflammatory Bowel ◽

Direct Peroral Cholangioscopy ◽

Secondary Biliary Cirrhosis

Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic disease characterized by inflammation and fibrosis that may involve the entire biliary tree. The fibrosis causes diffuse narrowing of the intrahepatic and extrahepatic bile ducts, and the resulting biliary stasis leads to secondary biliary cirrhosis and associated complications. This review addresses PSC through its epidemiology, etiopathogenesis, diagnosis, differential diagnosis, management, complications, and prognosis. Figures show pouchoscopy, liver biopsy, and endoscopic retrograde cholangiopancreatography; direct peroral cholangioscopy visualization of cholangiocarcinoma; and algorithms depicting diagnosis of cholangiocarcinoma and screening for inflammatory bowel disease (IBD) in patients with PSC. Tables list characteristics of IBD in PSC, prevalence of antibodies in PSC, differential diagnosis of PSC, diagnostic approach in PSC, and risk of cancers in PSC. This review contains 6 highly rendered figures, 5 tables, and 91 references.

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A Gentleman with Anemia and Cholestasis

Case Reports in Medicine ◽

10.1155/2010/536207 ◽

2010 ◽

Vol 2010 ◽

pp. 1-4

Author(s):

Siu-Tong Law ◽

Wai-Ki Lee ◽

Michael Kin-Kong Li ◽

Ka-Ho Lok

Keyword(s):

Inflammatory Bowel Disease ◽

Portal Hypertension ◽

Bile Duct ◽

Primary Sclerosing Cholangitis ◽

Bowel Disease ◽

Sclerosing Cholangitis ◽

Bile Ducts ◽

Colonoscopic Surveillance ◽

Extrahepatic Bile Ducts ◽

Inflammatory Bowel

Primary sclerosing cholangitis is a rare cause of cholestasis caused by progressive inflammation and fibrosis of both intrahepatic and extrahepatic bile ducts leading to multifocal ductal strictures. Herein, we report a case of primary sclerosing cholangitis and inflammatory bowel disease. The concomitant diagnosis of these two diseases is not typical. The management includes the treatment of inflammatory bowel disease and potential complications of primary sclerosing cholangitis, including dominant strictures of bile duct, portal hypertension, gallbladder diseases, cholangiocarcinoma, and colonoscopic surveillance.

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