Abstract
A 77-year-old man with jaundice was referred to our hospital. We found elevated level of serum hepatobiliary enzymes, IgG, and IgG4. Imaging studies demonstrated a pancreas head mass, segmental narrowing of the main pancreatic duct with an irregular wall in the pancreas head, and strictures of the intrahepatic and extrahepatic bile duct. His major duodenal papilla was swollen on duodenoscopy during endoscopic retrograde cholangiopancreatography, and infiltration of abundant IgG4-positive plasma cells was detected in the biopsy specimens taken from the swollen papilla. We diagnosed IgG4-related sclerosing cholangitis with autoimmune pancreatitis. After steroid therapy, his jaundice and the swelling of the pancreatic head were relieved and the swollen papilla with the abundant infiltration of IgG4-positive plasma cells disappeared.
