Abstract
A 59-year-old woman developed icterus and pruritus. She had a history of liver dysfunction, and on US a thickned gallbladder wall and stones in the neck of gallbladder were noted. ERCP showed stenosis of the right hepatic duct starting from the common biliary duct ; 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) showed focally increased FDG uptake. Surgery was performed given the diagnosis of Mirizzi syndrome-complex and the possibility of biliary tract cancer. A cholecystectomy, choledochectomy, and a hepaticoenterostomy were performed. Histological findings showed only inflammatory changes without any signs of malignancy. The patient was discharged but had to be readmitted sixth months later due to the presence of jaundice. Although percutaneous transhepatic cholangiography (PTC) showed stenosis and sclerosis of the right hepatic duct and the intrahepatic bile duct, drainage was successful in alleviating her jaundice. Based on immunohistochemical studies of a postoperative liver biopsy specimen and of a salivary gland biopsy specimen a final diagnosis of IgG4-related sclerosing cholangitis was made. The patient is now well controlled with steroids ; there have been no recrudescences.
