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): 2510-2515
Published online Mar 16, 2022. doi: 10.12998/wjcc.v10.i8.2510
Immunoglobulin G4-related kidney disease involving the renal pelvis and perirenal fat: A case report
Jun-Wei He, Qian-Ming Zou, Jun Pan, Shu-Sheng Wang, Song-Tao Xiang
Jun-Wei He, Qian-Ming Zou, Jun Pan, Shu-Sheng Wang, Song-Tao Xiang, Department of Urology, the Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510000, Guangdong Province, China
Author contributions: He JW drafted the manuscript and designed the figures and table; Zou QM and Wang SS analyzed and interpreted the data regarding computed tomography and magnetic resonance imaging; Pan J searched and reviewed the literature; Xiang ST participated in the final diagnosis and reviewed the final version of this work.
Supported by the National Natural Science Foundation of China, No. 8177140433.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: All authors have no conflicts of interest to report.
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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: Song-Tao Xiang, PhD, Chief Doctor, Department of Urology, the Second Affiliated Hospital of Guangzhou University of Chinese Medicine, No. 111 Dade Road, Yuexiu District, Guangzhou 510000, Guangdong Province, China. tonyxst@163.com
Received: July 22, 2021
Peer-review started: July 22, 2021
First decision: October 22, 2021
Revised: October 30, 2021
Accepted: February 10, 2022
Article in press: February 10, 2022
Published online: March 16, 2022
Processing time: 231 Days and 17 Hours
Abstract
BACKGROUND

Immunoglobulin (Ig) G4-related disease (IgG4-RD) is an autoimmune disease associated with chronic and progressive inflammation and fibrosis. It is difficult to differentiate IgG4-RD involving the kidney from infectious diseases and malignancy on imaging.

CASE SUMMARY

We report the case of a 51-year-old Chinese man whose abdominal computed tomography scan showed diffuse bilateral enlargement of the kidneys and perirenal fat, thickening of the renal pelvic walls, and hydronephrosis of the right kidney. Relevant laboratory test results showed a serum creatinine level of 464 μmol/L. The patient was diagnosed with acute renal failure and was started on intermittent hemodialysis. Further tests revealed high serum IgG4 levels (20.8 g/L) and an enlarged right submaxillary lymph node. Biopsy and histopathological examination of the enlarged node led to the diagnosis of IgG4-RD. After corticosteroid therapy, his serum creatinine level quickly decreased to near normal levels.

CONCLUSION

IgG4-RD affecting the renal pelvis or perirenal fat is rare, with atypical imaging features. Multidisciplinary consultation is critical for accurate diagnosis and treatment of this disease. Suspected cases should undergo biopsy to avoid misdiagnosis.

Keywords: Immunoglobulin G4-related disease; Renal pelvic; Perirenal fat; Case report

Core Tip: In this case, computed tomography showed diffuse bilateral enlargement of the kidneys and perirenal fat, thickening of the renal pelvic walls, and hydronephrosis of the right kidney which was similar to those of infectious diseases and malignancy. Histological examination confirmed the diagnosis of IgG4-RD.