Address of Corresponding Author

Pancreatology 2005;5:86-91 (DOI: 10.1159/000084494)

  Key Words

• Autoimmune pancreatitis
• Sclerosing cholangitis
• Retroperitoneal fibrosis
• Sjögren's syndrome
• IgG4
• Autoantibody
• Antinuclear antibody
• Antilactoferrin antibody

  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.

Copyright © 2005 S. Karger AG, Basel and IAP

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  Author Contacts

Kazuichi Okazaki
The Third Department of Internal Medicine
Kansai Medical University
10-15 Fumizono-cho, Moriguchi City, Osaka, 570-8506 (Japan)
Tel. +81 6 6992 1001, Fax +81 6 6996 4874, E-Mail okazaki@takii.kmu.ac.jp

  Article Information

Published online: March 16, 2005
Number of Print Pages : 6
Number of Figures : 4, Number of Tables : 1, Number of References : 32

  Publication Details

Pancreatology

Vol. 5, No. 1, Year 2005 (Cover Date: 2005)

Journal Editor: M.V. Singer, Mannheim; C.W. Imrie, Glasgow
ISSN: 1424-3903 (print), 1424-3911 (Online)

For additional information: http://www.karger.com/pan

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