A Rare Case of Small Duct Primary Sclerosing Cholangitis.

Background/Aims Small duct primary sclerosing cholangitis is a rare clinical condition that may remain unrecognized. Diagnosis rests on clinical, biochemical, and histologic features consistent with primary sclerosing cholangitis but with normal cholangiogram findings. We report here a case of small duct primary sclerosing cholangitis in a patient who presented with jaundice. Methods Clinical Presentation. A 22-year-old Filipino gentleman with no known comorbids came in due to a 2-week history of jaundice with elevated transaminases, bilirubin and alkaline phosphatase. No other symptoms were noted. Hepatitis markers were all normal. Physical examination was unremarkable except for jaundice. Results Management. Magnetic resonance cholangiopancreatography was normal with no evidence of dilated intra- and extrahepatic ducts. Liver biopsy showed intrahepatic cholestasis with periductal fibrosis. Ursodeoxycholic acid was started which showed significant decrease in jaundice with improvement in biochemistries. Conclusions Recommendation. Small duct primary sclerosing cholangitis appears to have a more favorable prognosis than classic primary sclerosing cholangitis. However, further studies with long-term follow up are recommended to determine disease progression. [ABSTRACT FROM AUTHOR]