Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jan 28, 2015; 21(4): 1334-1343
Published online Jan 28, 2015. doi: 10.3748/wjg.v21.i4.1334
Isolated intrapancreatic IgG4-related sclerosing cholangitis
Takahiro Nakazawa, Yushi Ikeda, Yoshiaki Kawaguchi, Hirohisa Kitagawa, Hiroki Takada, Yutaka Takeda, Isamu Makino, Naohiko Makino, Itaru Naitoh, Atsushi Tanaka
Takahiro Nakazawa, Itaru Naitoh, Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya 467-8601, Japan
Yushi Ikeda, Naohiko Makino, Department of Gastroenterology, Yamagata University Faculty of Medicine, Yamagata 990-9585, Japan
Yoshiaki Kawaguchi, Department of Gastroenterology, Tokai University School of Medicine, Kanagawa 259-1153, Japan
Hirohisa Kitagawa, Isamu Makino, Gastroenterologic Surgery, Division of Cancer Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa 920-8640, Japan
Hiroki Takada, Department of Gastroenterology, Kasugai Municipal Hospital, Kasugai 486-8510, Japan
Yutaka Takeda, Department of Surgery, Kansai Rosai Hospital, Osaka 660-8511, Japan
Atsushi Tanaka, Department of Medicine, Teikyo University School of Medicine, Tokyo 173-8606, Japan
Author contributions: Nakazawa T analyzed data and wrote the paper; Ikeda Y collected clinical data; Kawaguchi Y collected clinical data; Kitagawa H collected clinical data; Takada H collected clinical data; Takeda Y collected clinical data; Makino I collected clinical data; Makino N collected clinical data; Naitoh I analyzed data and wrote the paper; and Tanaka A analyzed data.
Supported by Health Labor Science Research Grants from Research on Measures for Intractable Diseases, the Intractable Hepato-Biliary Diseases Study Group in Japan.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Takahiro Nakazawa, MD, Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8601, Japan. tnakazaw@med.nagoya-cu.ac.jp
Telephone: +81-52-8538211 Fax: +81-52-8520952
Received: June 20, 2014
Peer-review started: June 22, 2014
First decision: July 9, 2014
Revised: July 29, 2014
Accepted: September 5, 2014
Article in press: September 5, 2014
Published online: January 28, 2015
Processing time: 220 Days and 21.5 Hours
Abstract

Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is frequently associated with type 1 autoimmune pancreatitis (AIP). Association with AIP can be utilized in the diagnosis of IgG4-SC. However, some cases of IgG4-SC are isolated from AIP, which complicates the diagnosis. Most of the reported cases of isolated IgG4-SC displayed hilar biliary strictures, whereas isolated IgG4-SC with intrapancreatic biliary stricture is very rare. Recently, we have encountered 5 isolated intrapancreatic IgG4-SC cases that were not associated with AIP, three of which were pathologically investigated after surgical operation. They all were males with a mean age of 74.2 years. The pancreas was not enlarged in any of these cases. No irregular narrowing of the main pancreatic duct was found. Bile duct wall thickening in lesions without luminal stenosis was detected by abdominal computed tomography in all five cases, by endoscopic ultrasonography in two out of four cases and by intraductal ultrasonography in all three cases. In three cases, serum IgG4 levels were within the normal limits. The mean serum IgG4 level measured before surgery was 202.1 mg/dL (4 cases). Isolated intrapancreatic IgG4-SC is difficult to diagnose, especially if the IgG4 level remains normal. Thus, this type of IgG4-SC should be suspected in addition to cholangiocarcinoma and pancreatic cancer if stenosis of intrapancreatic bile duct is present.

Keywords: Immunoglobulin G4-related sclerosing cholangitis; Isolated immunoglobulin G4-related sclerosing cholangitis; Autoimmune pancreatitis

Core tip: If stenosis of intrapancreatic bile duct is present and no abnormal findings of pancreas are detected, cholangiocarcinoma is suspected. Recently, we have encountered 5 isolated intrapancreatic immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) cases that were not associated with autoimmune pancreatitis, three of which were pathologically investigated after surgical operation. Isolated intrapancreatic IgG4-SC is difficult to diagnose, especially if the IgG4 level remains normal. Thus, this type of IgG4-SC should be suspected in addition to cholangiocarcinoma and pancreatic cancer if stenosis of intrapancreatic bile duct is present.