Tadashi Toyohara, Itaru Naitoh, Go Asano, Naruomi Jinno, Michihiro Yoshida, Yasuki Hori, Akihisa Kato, Kenta Kachi, Kayoko Oda, Kenta Saito, Yoichi Matsuo, Kenji Notohara, Hiromi Kataoka
We report a case of a 79-year-old woman with pancreaticobiliary maljunction (PBM) associated with IgG4-related sclerosing cholangitis (IgG4-SC) and type 1 autoimmune pancreatitis (AIP). Computed tomography revealed proximal bile duct dilatation and skip lesions in the head and tail of the pancreas. Endoscopic retrograde cholangiopancreatography revealed a long common channel and an intrapancreatic bile duct stricture. We preoperatively diagnosed distal bile duct cancer associated with PBM and AIP based on atypical biopsy findings and subsequently performed pancreaticoduodenectomy. The surgical specimen revealed IgG4-SC and AIP, without evidence of malignancy. To our knowledge, this is the first reported case of PBM associated with IgG4-SC and AIP in the literature.