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Korean J Pancreas Biliary Tract > Volume 17(1):2012 > Article
The Korean Journal of Pancreas and Biliary Tract 2012;17(1):23-28.
IgG4 연관 자가면역성 췌장염과 후복막 섬유증 1예
김미진, 한정호, 박우리, 최영락, 김병철, 장은영, 박찬선, 박선미
충북대학교 의과대학 내과학교실
A Case of IgG4-Related Autoimmune Pancreatitis and Retroperitoneal Fibrosis
Mi Jin Kim, Joung-Ho Han, Woo Ri Park, Young Rak Choi, Byung Chul Kim, Eun Young Jang, Chan Sun Park, Seon Mee Park
Department of Internal Medicine, Chungbuk National University College of Medicine, Cheongju, Korea
ABSTRACT
IgG4-related systemic diseases include autoimmune pancreatitis and a variety of extrapancreatic lesions, including retroperitoneal fibrosis, sclerosing cholangitis, and tubulointerstitial nephritis. This report describes a case of IgG4-related autoimmune pancreatitis and recurrent retroperitoneal fibrosis, from which the patient recovered via steroid therapy. A 70-year-old female presented with abdominal pain and jaundice. She had been treated with prednisolone for recurrent retroperitoneal fibrosis for 3 years. An abdominal computed tomography demonstrated bilateral hydronephrosis caused by retroperitoneal fibrosis, intra- and extra-hepatic bile duct dilatation and diffuse pancreas enlargement. Endoscopic Retrograde Cholangio- Pancreatography showed intrapancreatic bile duct stenosis and IgG4 stained cell infiltration was observed on biopsy tissues of the duodenal papilla and retroperitoneum. After 3 months of steroid treatment, bile duct stenosis, pancreas enlargement, and retroperitoneal fibrosis improved. Also, an elevated serum IgG4 level returned to the normal range, and IgG4-positive plasma cells disappeared from the follow-up biopsy tissues of the duodenal papilla.
Keywords: Retroperitoneal fibrosis, Autoimmune pancreatitis, IgG4, IgG4-related systemic disease
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