Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Mar 16, 2022; 10(8): 2543-2549
Published online Mar 16, 2022. doi: 10.12998/wjcc.v10.i8.2543
Immunoglobulin G4-related disease involving multiple systems: A case report
Yu-Qiong An, Ning Ma, Yong Liu
Yu-Qiong An, Ning Ma, Heart Center, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing 100045, China
Yong Liu, Department of Ultrasound, Beijing-Shijitan Hospital, Capital Medical University, Beijing 100038, China
Author contributions: An YQ reviewed the literatures and contributed to manuscript drafting; Liu Y analyzed and interpreted the imaging findings; Liu Y and Ma N were responsible for the revision of the important intellectual contents; all authors issued the final approval for the submitted version.
Supported by Beijing Hospitals Authority Clinical Medicine Development of special funding support, No. XMLX202113; Beijing Municipal Science &Technology Commission, No. Z191100006619051; Special Fund for Open Topics of Lymph Surgery in Beijing Shijitan Hospital Affiliated to Capital Medical University, No. 2019-LB03; and Beijing Natural Science Foundation, No. 7192062.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have 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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Yong Liu, MD, PhD, Chief Doctor, Professor, Department of Ultrasound, Beijing-Shijitan Hospital, Capital Medical University, Tieyi Road, Yangfangdian, Beijing 100038, China. liuy@bjsjth.cn
Received: August 16, 2021
Peer-review started: August 16, 2021
First decision: November 6, 2021
Revised: November 16, 2021
Accepted: January 27, 2022
Article in press: January 27, 2022
Published online: March 16, 2022
Processing time: 206 Days and 15.5 Hours
Abstract
BACKGROUND

IgG4-related disease (IgG4-RD), an immune-mediated chronic progressive fibroinflammatory disease, can affect the functions of several organs. Some common characteristics can be observed in different IgG4-RDs, such as higher prevalence in middle-aged and elderly male patients, raised serum IgG4 levels, abundant infiltration of IgG4-positive cells and fibrosis, diffuse or localized swelling of the affected organs, and good response to glucocorticoids treatment.

CASE SUMMARY

A 72-year-old man complained of left upper abdominal pain 3 mo ago, and he was diagnosed with acute onset of chronic cholecystitis and acute pancreatitis in the local hospital. Pain improved after relevant treatment. Several days ago, his abdominal pain worsened, and he was admitted to our hospital for further treatment. Doppler ultrasound showed that the pancreas presented with sausage-like swelling and the parenchymal echo was diffusely reduced. Gallbladder volume was increased, while the wall was rough and thickened with bilateral signs. Furthermore, the left submandibular gland was enlarged, accompanied with significantly increased blood flow signals. Finally, we found that the adventitia of the abdominal aorta and right iliac artery was thickened locally. Serum IgG4 was elevated to 12600 mg/L. Therefore, the patient was diagnosed with IgG4-RD. After treatment with methylprednisolone, he had an uneventful course and was discharged in good condition.

CONCLUSION

IgG4-RD can involve almost any organs. Ultrasound has a significant role in timely and accurately diagnosis.

Keywords: Immunoglobulin G4; Autoimmune pancreatitis; Cholecystitis; Arteritis; Ultrasound; Case report

Core Tip: Immunoglobulin G4-related disease (IgG4-RD) can involve multiple organs and sites, such as the glands and ductal tissues. However, it is rare that a patient with more than three organs are involved at the same time, as well as the arterial lesions. In our case, we report a patient with the autoimmune inflammation, his four organs and tissues are involved, they are the aorta, pancreas, gallbladder, and submandibular gland. Meanwhile, the involved abdominal aorta and iliac artery presented as IgG4-related periarteritis. Finally, we made a comprehensive diagnosis according to the clinical histology, imaging, serology, and the response to the therapy.