A Case of Autoimmune Pancreatitis Associated with Sclerosing Cholangitis, Retroperitoneal Fibrosis and Sjögren’s Syndrome

Vol. 5, No. 1, 2005

Free Abstract Article (References) Article (PDF 536 KB)
Free Access

Case Report

A Case of Autoimmune Pancreatitis Associated with Sclerosing Cholangitis, Retroperitoneal Fibrosis and Sjögren's Syndrome
Toshiro Fukui^a, Kazuichi Okazaki^b, Hazuki Yoshizawa^a, Shinya Ohashi^a, Hiroyuki Tamaki^a, Kimio Kawasaki^a, Minoru Matsuura^a, Masanori Asada^a, Hiroshi Nakase^a, Yasuaki Nakashima^c, Akiyoshi Nishio^a, Tsutomu Chiba^a

^aDepartment of Gastroenterology and Endoscopic Medicine, Graduate School of Medicine, Kyoto University, Kyoto,
^bThe Third Department of Internal Medicine, Kansai Medical University, Moriguchi,
^cDepartment of Pathology, Kyoto University Hospital, Kyoto, Japan

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.

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

Medline Abstract (ID 15775703)

Download Citation

Cited In

For non-native English speakers and international authors who would like assistance with their writing before submission, we suggest American Journal Experts for their scientific editing service.