IgG-4-Related Gallbladder Disease Mimicking Mirizzi Syndrome

A woman in her sixth decade was transferred to our tertiary hospital for management of obstructive jaundice and concern for a polypoid gallbladder mass. Magnetic resonance cholangiopancreatography demonstrated a 3.7 cm gallstone in the gallbladder neck, thickened gallbladder wall and common hepatic duct stricture adjacent to the stone with resultant intrahepatic biliary ductal dilation suggesting a diagnosis of Mirizzi syndrome versus neoplasia. Endoscopic retrograde cholangiopancreatography showed a severe biliary stricture and a stent was placed to span the stricture. Cytology brushings of the stricture were negative for malignant cells. Surgical pathology from a diagnostic laparoscopy converted to an open partial cholecystectomy revealed a histological diagnosis of IgG4 related cholecystitis. Following hospital discharge, the patient was referred to our gastroenterology clinic for evaluation of IgG4 cholangiopathy with magnetic resonance cholangiopancreatography evidence of persistent biliary stricture post-operatively. The patient was successfully treated with steroid therapy, as follow-up imaging demonstrated resolution of biliary dilation and improvement in the common hepatic duct stenosis and normalization of serum bilirubin and alkaline phosphatase. Received September 26th, 2016 Accepted November 05th, 2016