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Copyright ©2008 The WJG Press and Baishideng. All rights reserved.
World J Gastroenterol. Jul 7, 2008; 14(25): 3974-3981
Published online Jul 7, 2008. doi: 10.3748/wjg.14.3974
Primary sclerosing cholangitis - What is the difference between east and west?
Ali Shorbagi, Yusuf Bayraktar
Ali Shorbagi, Yusuf Bayraktar, Hacettepe University, School of Medicine, Department of Internal Medicine, Gastroenterology Clinic, Sihhiye 06100, Ankara, Turkey
Author contributions: Shorbagi A and Bayraktar Y both contributed to this paper; Bayraktar Y is the main physician, responsible for patients with primary sclerosing cholangitis at Hacettepe University; Shorbagi A did the research necessary to make this manuscript possible; and Shorbagi A and Bayraktar Y co-wrote this review.
Correspondence to: Ali Shorbagi, MD, Hacettepe University, School of Medicine, Department of Internal Medicine, Gastroenterology clinic, Sihhiye 06100, Ankara, Turkey. shorbagi@hacettepe.edu.tr
Telephone: +90-555-2197557
Fax: +90-312-3051480
Received: February 19, 2008
Revised: May 21, 2008
Accepted: May 28, 2008
Published online: July 7, 2008
Abstract

Primary sclerosing cholangitis (PSC) is a chronic, progressive, cholestatic liver disease characterized by inflammation and fibrotic obliteration of the hepatic biliary tree. It is commonly associated with inflammatory bowel disease (IBD). A number of complications can occur which require special consideration, the most important of which is the development of cholangiocellular carcinoma (CCC). Unfortunately, no medical therapy is currently available for the underlying liver disease. Liver transplantation is an effective, life-extending option for patients with advanced PSC. Geographical variations between East and West include a second peak for age with a lower association with IBD in a Japanese population and female predominance in a lone study from Turkey. The clinical and biochemical Mayo criteria may not be universally applicable, as different patients show variations regarding the initial presentation and natural course of the disease. Directing research towards explaining these geographical differences and understanding the pathogenesis of PSC is required in order to develop better therapies for this devastating disease.

Keywords: Primary sclerosing cholangitis, Endoscopic retrograde cholangiopancreatography, Magnetic resonance cholangiopancreatography, Cholangiocellular carcinoma, Ursodeoxycholic acid