Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jan 6, 2021; 9(1): 267-273
Published online Jan 6, 2021. doi: 10.12998/wjcc.v9.i1.267
Immunosuppressant treatment for IgG4-related sclerosing cholangitis: A case report
Jong-Sun Kim, Won Ho Choi, Kyung-Ann Lee, Hyun-Sook Kim
Jong-Sun Kim, Won Ho Choi, Kyung-Ann Lee, Hyun-Sook Kim, Department of Internal Medicine, Soonchunhyang University Seoul Hospital, Seoul 04401, South Korea
Author contributions: Kim JS, Kim HS contributed to conceptualization; Choi WH, Lee KA contributed to data curation; Kim JS, Lee KA contributed to writing-original draft; Kim JS, Kim HS contributed to writing review and editing; and all authors have read and approve the final manuscript.
Supported by Soonchunhyang University.
Informed consent statement: We provided informed written consent prior to study enrollment.
Conflict-of-interest statement: This study was supported by Soonchunhyang University. There is no conflict-of-interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
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: Hyun-Sook Kim, MD, PhD, Full Professor, Department of Internal Medicine, Soonchunhyang University Seoul Hospital, 59 Daesagwanro Youngsangu, Seoul 04401, South Korea. healthyra@schmc.ac.kr
Received: October 1, 2020
Peer-review started: October 1, 2020
First decision: November 3, 2020
Revised: November 9, 2020
Accepted: November 21, 2020
Article in press: November 21, 2020
Published online: January 6, 2021
Abstract
BACKGROUND

Immunoglobulin G4-related disease (IgG4-RD) is a multi-system fibroin-flammatory disorder that can involve any organ, including the salivary glands, pancreas, and biliary tree. Treatment of immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is similar to that for IgG4-RD, but progression is irreversible in some cases. We present a case of IgG4-SC in which an immuno-suppressant induced marked clinical and radiologic improvement.

CASE SUMMARY

A 63-year-old male presented with a prominent itching sensation and wholebody jaundice. He showed obstructive-pattern jaundice, an elevated IgG4 level, and infiltration of a large number of IgG4-positive cells in the ampulla of Vater. The imaging findings of intrahepatic duct (IHD) and common bile duct dilation, an elevated serum IgG4 level, and characteristic histological findings led to diagnosis of IgG4-SC that compatible with the 2019 ACR/EULAR classification criteria. We planned to treat the patient with high-dose glucocorticoid (GC), followed by cyclophosphamide pulse therapy. After treatment with high-dose GC and an immunosuppressant, imaging studies showed that IHD dilatation had completely resolved.

CONCLUSION

Prompt diagnosis and appropriate treatment of IgG4-SC are important. Because there is a risk of relapse of IgG4-SC, the GC dose should be gradually reduced, and a maintenance immunosuppressant should be given.

Keywords: Immunoglobulin G4-related disease, Immunoglobulin G4-related sclerosing cholangitis, Glucocorticoid, Immunosuppressant, Case report, ACR/EULAR classification criteria

Core Tip: In this paper, we presented immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) which was diagnosed by ACR/EULAR published new classification criteria and was successfully treated using a glucocorticoid with an immunosuppressant and made approved as imaging follow-up. Prompt diagnosis and appropriate treatment of IgG4-SC are important to induce rapid regression and to prevent irreversible fibrosis and biliary stricture. This case emphasizes the proactive attitude to use immuno-suppressants in treatment with IgG4-SC.