Primary sclerosing cholangitis--what is the difference between east and west?
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.
Shorbagi
Ali
A
Hacettepe University, School of Medicine, Department of Internal Medicine, Gastroenterology clinic, Sihhiye 06100, Ankara, Turkey. shorbagi@hacettepe.edu.tr
Bayraktar
Yusuf
Y
D000328:Adult; D044466:Asian People; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000208:ethnology* / Q000209:etiology / Q000473:pathology / Q000628:therapy; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000208:ethnology; D007564:Japan / Q000453:epidemiology; D016031:Liver Transplantation; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D016896:Treatment Outcome; D044465:White People