Primary Sclerosing Cholangitis: From Pathogenesis to Medical Management [Abstract]

[N A J Med Sci. 2012;5(2):82-93.] PDF File

Chalermrat Bunchorntavakul, MD; Tawesak Tanwandee, MD;
Phunchai Charatcharoenwitthaya, MD; K. Rajender Reddy, MD*

Primary sclerosing cholangitis (PSC) is a cholestatic  liver disease characterized by progressive inflammatory destruction of intrahepatic and extrahepatic  bile ducts. It is strongly associated with inflammatory bowel disease, particularly ulcerative  colitis. The pathogenesis of PSC remains unclear, however several hypotheses have been proposed  that suggest roles for autoimmunity, genetic susceptibility, and the interaction between microorganisms  and host immune response directed at the biliary system. A diagnosis of PSC is based on a  constellation of clinical, biochemical, and typical cholangiographic features and usually  without the need for liver histopathology. Complications of PSC include pruritus,  portal hypertension, bone disease, end-stage liver disease, and cancers. Cholangiocarcinoma  eventually develops in 8-15% of PSC patients. A variety of drugs have been evaluated as therapy  for PSC, but no therapy has yet been proven to prolong survival or improve outcomes in PSC.  Ursodeoxycholic acid (UDCA) has been intensively investigated to address its efficacy in  PSC. A recent investigation noted that high-dose UDCA therapy in PSC did not confer benefit  on combined clinical and survival endpoints. . Immunosuppressive agents are generally ineffective.  Liver transplantation remains the only proven long-term treatment for advanced PSC,  with approximately 20-25% risk of disease recurrence. Cancer surveillance, management of  cirrhotic complications, and treatment of manifestations of cholestasis in those with PSC  are clinically relevant. Further understanding of the pathogenesis of PSC is desperately required  in order to effectively improve our current approaches to the management of this disease. 

Key Words: primary sclerosing cholangitis, cholestasis, pruritus, inflammatory bowel primary sclerosing cholangitis, cholestasis, pruritus, inflammatory bowel disease, ulcerative colitis, pathogenesis, management, cholangiocarcinoma

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Chalermrat Bunchorntavakul, MD;1,3 Tawesak Tanwandee, MD;2 Phunchai Charatcharoenwitthaya, MD;2 K. Rajender Reddy, MD3*

1Division  of Gastroenterology and Hepatology, Department of Medicine, Rajavithi Hospital, College of  Medicine, Rangsit University, Bangkok, Thailand
2Division of Gastroenterology and  Hepatology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University,  Bangkok, Thailand
3Division of Gastroenterology and Hepatology, Department of  Medicine, University of Pennsylvania, Philadelphia, PA 

*Corresponding Author: Professor  of Medicine, Hospital of the University of Pennsylvania, 2 Dulles, 3400 Spruce Street,  Philadelphia, PA 19104, USA. Tel: 215-662-4311. (Email: 

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