312 • Hepatobiliary Pancreat Dis Int，Vol 8，No 3 • June 15，2009 • www.hbpdint.com Author Affiliations: Department of Gastroenterology, Türkiye Yüksek İhtisas Hospital, Ankara, Turkey (Ataseven H, Parlak E, Ertuğrul İ, Şaşmaz N and Şahin B); Department of Gastroenterology, Dişkapı Yildirım Beyazıt Education and Research Hospital, Ankara, Turkey (Yüksel İ and Başar Ö) Corresponding Author: İlhami Yüksel, MD, Specialist, Uyaniş Mahallesi, Kızlarpınarı Caddesi No: 278/9, Keciören, Ankara, Türkiye (Tel: +90-312-3817153; Fax: +90-312-3186690; Email: yukselilhami@ hotmail.com) © 2009, Hepatobiliary Pancreat Dis Int. All rights reserved. BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by destruction and fibrosis of the bile ducts. This study aimed to demonstrate the hepatic and extrahepatic characteristic findings and prognostic outcomes of Turkish patients with PSC. METHODS: The medical records of 35 consecutive patients with PSC from January 1988 to June 2007 were recorded prospectively. From the time of diagnosis, clinical features and laboratory data were collected. RESULTS: The mean age of the 35 patients was 41.69 years (range 15-80 years) at the time of diagnosis; 14 (40%) were female, and 21 (60%) were male. The mean duration of follow-up was 58.86 months (1-180 months). Twenty (57.1%) of the patients with PSC were asymptomatic and 22 (62.9%) had inflammatory bowel disease. At the time of diagnosis, 20 (57.1%) of the patients had both intra- and extra-hepatic PSC. Twenty-one (60%) of the patients, who had undergone ERCP for stent placement, had dominant bile duct stenosis. Cholangiocarcinoma was found in 2 (5.7%) of the patients and cirrhosis was detected in 7 (20%); 5 (14.3%) underwent liver transplantation. The median follow-up time after liver transplantation was 23 months and all are still alive. Six (17.1%) patients died. CONCLUSIONS: PSC has a clinical course varied from advanced liver disease requiring liver transplantation within a short time to being asymptomatic for decades. The prognosis of Turkish patients with PSC is also disappointing as described in other studies. (Hepatobiliary Pancreat Dis Int 2009; 8: 312-315) KEY WORDS: primary sclerosing cholangitis; cholestatic liver diseases; biliary tree; endoscopic retrograde cholangiopancreatography Introduction P rimary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by destruction and fibrosis of the bile ducts. Diagnostic criteria are based on the characteristic changes in the intra- and extra-hepatic biliary tree as seen with endoscopic retrograde cholangiopancreatography (ERCP) or percutaneous transhepatic cholangiography (PTC). [1, 2] Invasive imaging modalities such as ERCP and PTC, and non-invasive techniques such as magnetic resonance cholangiopancreatography, have led to more frequent diagnosis of PSC. PSC is a rare disease with a prevalence of 3.8-13 per 100 000 persons. [3, 4] In a recent study, the inci- dence of PSC was found to be 0.41 per 100 000 in a European population-based cohort study. [4] The clinical course is varied, endoscopic and medical treatment choices are present, but the disease is usually progressive and can progress to cirrhosis. [5] Biliary ductal inflammation and fibrosis progress to cholangiocarcinoma (8%-10%), and ethnic differences can affect the prognosis. [6-8] The only life-extending treatment choice for patients with a poor prognosis of PSC is liver transplantation. [9] The outcome of patients with PSC in Turkey has not been described. This study demonstrated the hepatic and extrahepatic characteristic findings and prognostic outcomes of Turkish patients with PSC. Methods Patients Primary sclerosing cholangitis in Turkish patients: characteristic features and prognosis Hilmi Ataseven, Erkan Parlak, İlhami Yüksel, Ömer Başar, İbrahim Ertuğrul, Nurgül Şaşmaz and Burhan Şahin Ankara, Turkey Original Article / Biliary