Clinical profile of primary sclerosing cholangitis in Singapore

BACKGROUND AND AIM: Primary sclerosing cholangitis (PSC) is a rare chronic disease in Singapore and its epidemiological profile has not been described previously. The present study aimed to define the demographic and clinical profile of patients with PSC in Singapore. METHODS: The case records of patients with PSC seen at Changi General Hospital were analyzed in terms of demographic profile, clinical presentation, clinical course, treatment and complications. RESULTS: Ten cases of PSC were diagnosed over a 10-year period. The male:female ratio was 9:1. The median age of diagnosis was 49.5 years (mean: 50.9 years; range: 35-63 years). With regards to clinical presentation, seven patients had hepatobiliary sepsis, two patients had asymptomatic liver biochemistry abnormalities while one patient had cholestatic jaundice. Prevalence rate of perinuclear antineutrophil cytoplasmic antibody (pANCA) was 20%. Symptomatic inflammatory bowel disease (IBD) was diagnosed in 20% of PSC cases. Eight patients (80%) had intrahepatic ductal involvement while two patients (20%) had combined intrahepatic and extrahepatic ductal involvement on endoscopic retrograde cholangiopancreatography (ERCP). The prevalence rate of recurrent cholangitis was 30% while that of recurrent liver abscess, cirrhosis and common bile duct stricture were all 10%. The mean duration of follow up was 6.6 years with one death from liver failure. CONCLUSION: The clinical profile of patients with PSC in Singapore appears to differ with other published data, with a greater number presenting with hepatobiliary sepsis and less frequent association with IBD and pANCA. It is hypothesized that this may be related to differences in environmental triggers and genetic susceptibility.