Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Nov 26, 2020; 8(22): 5821-5830
Published online Nov 26, 2020. doi: 10.12998/wjcc.v8.i22.5821
Usefulness of ultrasonography to assess the response to steroidal therapy for the rare case of type 2b immunoglobulin G4-related sclerosing cholangitis without pancreatitis: A case report
Yuto Tanaka, Kenya Kamimura, Ryota Nakamura, Marina Ohkoshi-Yamada, Yohei Koseki, Takeshi Mizusawa, Satoshi Ikarashi, Kazunao Hayashi, Hiroki Sato, Akira Sakamaki, Junji Yokoyama, Shuji Terai
Yuto Tanaka, Kenya Kamimura, Ryota Nakamura, Marina Ohkoshi-Yamada, Yohei Koseki, Takeshi Mizusawa, Satoshi Ikarashi, Kazunao Hayashi, Hiroki Sato, Akira Sakamaki, Junji Yokoyama, Shuji Terai, Division of Gastroenterology and Hepatology, Graduate School of Medical and Dental Sciences, Niigata University, Niigata 9518510, Japan
Author contributions: Tanaka Y, Kamimura K, Terai S wrote the manuscript; Nakamura R, Ohkoshi-Yamada M, Koseki Y, Mizusawa T, Ikarashi S, Hayashi K, Sato H, Sakamaki A, Yokoyama J treated patients and performed histological analysis; all authors read and approved the final version of the manuscript.
Informed consent statement: Written informed consent was obtained from the patient for publication of this case report and the accompanying images.
Conflict-of-interest statement: The authors declare that they have no current financial arrangement or affiliation with any organization that may have a direct influence on their work.
CARE Checklist (2016) statement: CARE Checklist (2016) of information was included and the form was attached.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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/
Corresponding author: Kenya Kamimura, MD, PhD, Lecturer, Department of Division of Gastroenterology and Hepatology, Graduate School of Medical and Dental Sciences, Niigata University, 1-757 Asahimachido-ri, Chuo-ku, Niigata 9518510, Japan. kenya-k@med.niigata-u.ac.jp
Received: September 5, 2020
Peer-review started: September 5, 2020
First decision: September 30, 2020
Revised: September 30, 2020
Accepted: October 26, 2020
Article in press: October 26, 2020
Published online: November 26, 2020
Core Tip

Core Tip: Immunoglobulin G4-associated sclerosing cholangitis (IgG4-SC) is a biliary tract lesion of the systemic IgG4-related disease. It is often complicated with autoimmune pancreatitis, and if not related, it is diagnosed as an isolated IgG4-SC. The corticosteroid treatment shows the favorable response by improving the infiltration of inflammatory cells, wall thickness, and decrease the serum IgG4. With a variety of the types of IgG4-SC determined by cholangiogram, differential diagnosis from the malignancy and primary sclerosing cholangitis is essential. In the present study, we report a rare elderly and asymptomatic case of type 2b, isolated IgG4-SC who was differentially diagnosed from primary sclerosing cholangitis by liver biopsy. The administration of corticosteroid improved the inflammation, normalized the serum IgG4 Level, and improved the wall thickness of the bile duct. As isolated type 2b, IgG4-SC is rare, the images, histological findings, and clinical course of our case will be helpful for physicians to diagnose and treat the new cases appropriately.