Sclerosing cholangitis (SC) with granulocytic epithelial lesion (GEL) responds well to immunosuppression therapy. We treated a 42-year-old Japanese female with ulcerative colitis, who was admitted for further evaluation of both an elevated alkaline phosphatase level and dilated intrahepatic bile ducts. A liver biopsy on the fourth hospital day revealed the infiltration of neutrophils into the bile duct epithelium, which was diagnosed as GEL. Because her ulcerative colitis was in an active stage, prednisolone (PSL) therapy was started. After the administration of PSL, laboratory data dramatically decreased. A liver biopsy was performed on the 66th hospital day to confirm the lesion around bile ducts in the portal tract. The infiltration of neutrophils into the bile duct epithelium disappeared after PSL administration, and IgG4-positive plasma cells were not found in the liver. Herein, we report a rare case of GEL-positive SC. The present case provides early evidence of treatment-induced histological changes as well as serial changes in biochemical data during the course of immunosuppression therapy.
Keywords: GEL; PR3-ANCA; granulocytic epithelial lesion; sclerosing cholangitis.
© 2016 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd.