A case of primary sclerosing cholangitis with unique clinical features for a 5-year follow up

Abstract

A 26-year old young woman, who had been pointed out to have liver dysfunction in a medical check, admit-ted to our hospital in 2008. At first, she was slight obesity and suspected to be non-alcoholic fatty liver disease (NAFLD), because serology for hepatitis B virus, hepatitis C virus and various autoimmune markers were nega-tive in a screening blood examination. However, the hepatic dysfunction had continued after exercise and die-tary therapy, and even after administration of ursodeoxycholic acid and statin preparation. Liver biopsies per-formed in 2010 and 2012 did not lead to a definitive diagnosis, but localized high-echoic distinct lesions appeared in 2012 in her whole liver as diffuse lesions with a significant rise of the tumor marker in 2013. Therefore, we performed a liver biopsy to rule out hepato-biliary malignancies. The liver biopsy specimen did not show any malignant finding, but histological examination showed an extremely rapid progression of liver fibrosis. Cholangiographic imaging study such as magnetic resonance (MR) cholangiopancreatography and endo-scopic retrograde cholangiopancreatography revealed diffusely dilated intrahepatic ducts with multifocal nar-rowing with diffuse ductal wall thickening and enhancement and confirmed the diagnosis of primary sclerosing cholangitis (PSC). In this report, we describe an unique case of PSC with detailed histological features and imag-ing studies for a 5-year follow up.