title: A case of autoimmune pancreatitis associated with sclerosing cholangitis, retroperitoneal fibrosis and Sjögren's syndrome.
abstract: We report a very rare case of autoimmune pancreatitis (AIP) associated with sclerosing cholangitis, retroperitoneal fibrosis and Sjögren's syndrome. The patient had an enlarged pancreas, and autoantibodies were detected in the serum. Serum IgG and IgG4 concentrations were also elevated. Endoscopic retrograde cholangiopancreatography revealed an irregular narrowing of the main pancreatic duct from the head to the body and sclerotic change in the intrapancreatic common bile duct, which later extended to the intrahepatic bile ducts. In addition, histological examination of the liver revealed lymphocytic sclerosis around the bile ducts, similar to the histology in the pancreas of AIP. Retroperitoneal tumors were diagnosed as retroperitoneal fibrosis by histological examination. Serological and functional abnormalities suggestive of Sjögren's syndrome were detected, and histological findings of the lip were compatible with Sjögren's syndrome. Immunohistochemistry of each lesion disclosed that most of the infiltrating lymphocytes were T cells with similar levels of both CD4+ and CD8+ cells. Moreover, some of the infiltrating plasma cells were positive for anti-IgG4 monoclonal antibody. These diseases were dramatically improved by steroid therapy. Although the pathophysiology of AIP is still unclear, the present case suggests a common pathophysiological mechanism for AIP, sclerosing cholangitis, retroperitoneal fibrosis and Sjögren's syndrome.
authors.affiliation: Department of Gastroenterology and Endoscopic Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
authors.forename: Toshiro
authors.initials: T
authors.lastname: Fukui
authors.forename: Kazuichi
authors.initials: K
authors.lastname: Okazaki
authors.forename: Hazuki
authors.initials: H
authors.lastname: Yoshizawa
authors.forename: Shinya
authors.initials: S
authors.lastname: Ohashi
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Tamaki
authors.forename: Kimio
authors.initials: K
authors.lastname: Kawasaki
authors.forename: Minoru
authors.initials: M
authors.lastname: Matsuura
authors.forename: Masanori
authors.initials: M
authors.lastname: Asada
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Nakase
authors.forename: Yasuaki
authors.initials: Y
authors.lastname: Nakashima
authors.forename: Akiyoshi
authors.initials: A
authors.lastname: Nishio
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Chiba
mesh_terms: D000368:Aged; D001327:Autoimmune Diseases / Q000175:diagnosis; D015209:Cholangitis, Sclerosing / Q000175:diagnosis*; D006801:Humans; D008297:Male; D010195:Pancreatitis / Q000175:diagnosis* / Q000276:immunology; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis*; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000473:pathology