Abstract

Objective To investigate the differential diagnosis and management of IgG4 associated biliary pancreatic diseases. Methods A total of 18 patients with jaundice and abdominal pain were retrospectively studied. The final diagnosis was 7 cases of type Ⅰ autoimmune pancreatitis (Ⅰ-AIP), 4 cases of IgG4-associated sclerosing cholangitis (IgG4-SC), and 7 cases of I-AIP combined IgG4-SC. Results (1)Imaging features: I-AIP patients showed pancreatic head mass, with relatively poor blood supply when under dynamic contrast-enhanced scan, in delay image, enhanced amplitude more than the normal pancreas. IgG4-SC can be seen in patients with symmetrical bile duct wall stenosis and (or) soft tissue mass, the thickened bile duct wall and intraluminal soft tissue mass can be enhanced with bile duct wall thickening.(2)Serological characteristics: Serum IgG4 fluctuated between 59.3 and 1 120.0 mg/dl, CA19-9 fluctuated between 12.2 and 230.8 U/ml.(3)Histopathology: Plasma cells lymphocyte infiltration and fibrosis, immunohistochemical staining showed IgG4 positive cells >10/hpf, and obliterative phlebitis.(4)Combined with other organ lesions in 6 cases including renal injury, retroperitoneal fibrosis, mesenteric inflammatory nodules, omental inflammatory nodules, submandibular gland enlargementin, and pituitary inflammation . (5)Response to hormone therapy: 4 out of 6 operated patients were none resected and were given hormonotherapy . In twelve non-surgical patients, 2 cases abandoned therapy, 10 cases were treated with hormone therapy, and improved. Conclusion IgG4 associated biliary pancreatic disease characterized by localized pancreatic or bile duct disease, can be distinguished from malignancy by special imaging, IgG4 elevation, histopathology and positive response to experimental hormonotherapy . Key words: Autoimmune diseases; Pancreatic neoplasms; Bile duct neoplasms

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