He PH, Liu LC, Zhou XF, Xu JJ, Hong WH, Wang LC, Liu SJ, Zeng JH. IgG4-related kidney disease complicated with retroperitoneal fibrosis: A case report. World J Clin Cases 2023; 11(7): 1656-1665 [PMID: 36926395 DOI: 10.12998/wjcc.v11.i7.1656]
Corresponding Author of This Article
Li-Chang Liu, MD, Chief Doctor, Professor, Department of Nephrology, Zhuhai Hospital of Guangdong Provincial Hospital of Chinese Medicine, No. 53 Jida Jingle Road, Xiangzhou District, Zhuhai 519015, Guangdong Province, China. 1225929054@qq.com
Research Domain of This Article
Urology & Nephrology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
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/
Pei-Hua He, Xing-Fu Zhou, Jun-Jie Xu, The Second Clinical Medical College, Guangzhou University of Chinese Medicine, Guangzhou 510006, Guangdong Province, China
Li-Chang Liu, Wei-Hong Hong, Li-Chun Wang, Su-Jun Liu, Jia-Hao Zeng, Department of Nephrology, Zhuhai Hospital of Guangdong Provincial Hospital of Chinese Medicine, Zhuhai 519015, Guangdong Province, China
Author contributions: He PH contributed to manuscript writing and editing; Zhou XF contributed to data collection; Hong WH, Liu SJ, Wang LC, and Zeng JH contributed to data analysis; Xu JJ contributed to illustration; and Liu LC contributed to conceptualization and supervision. All authors have read and approved the final manuscript.
Supported bythe Introduction of High-level Health Team Project in Zhuhai.
Informed consent statement: Written informed consent was obtained from the patient for the publication of this report and any accompanying images.
Conflict-of-interest statement: All the authors declare that they have no conflicts of interest to disclose.
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: Li-Chang Liu, MD, Chief Doctor, Professor, Department of Nephrology, Zhuhai Hospital of Guangdong Provincial Hospital of Chinese Medicine, No. 53 Jida Jingle Road, Xiangzhou District, Zhuhai 519015, Guangdong Province, China. 1225929054@qq.com
Received: December 8, 2022 Peer-review started: December 8, 2022 First decision: January 3, 2023 Revised: January 11, 2023 Accepted: February 15, 2023 Article in press: February 15, 2023 Published online: March 6, 2023 Processing time: 84 Days and 2.1 Hours
Abstract
BACKGROUND
IgG4-related disease (IgG4-RD) is a chronic fibrotic disease mediated by immunity recognized by clinicians in recent years. When the kidney is involved, it is called IgG4-related kidney disease (IgG4-RKD). IgG4-related tubulointerstitial nephritis (IgG4-TIN) is a representative manifestation of IgG4-RKD. IgG4-TIN can cause obstructive nephropathy complicated by retroperitoneal fibrosis (RPF). Cases of IgG4-TIN complicated with RPF are rare. Glucocorticoids are the first-line therapeutic medication for IgG4-RD and can significantly improve renal function.
CASE SUMMARY
Herein, we report the case of a 56-year-old man with IgG4-RKD complicated with RPF. The patient presented to the hospital with complaints of elevated serum creatinine (Cr), nausea, and vomiting. During hospitalization, Cr was 1448.6 µmol/L, and serum IgG4 was increased. A total abdominal computed tomography (CT) scan and enhanced CT scan obviously indicated RPF. Although this patient had a long course and renal insufficiency, we performed a kidney biopsy. Renal biopsy showed that the renal tubulointerstitium had focal plasma cell infiltration and increased lymphocyte infiltration accompanied by fibrosis. After combining the biopsy results with immunohistochemistry, it was found that the absolute number of positive IgG4+ cells per high power field exceeded 10, and the ratio of IgG4/IgG was over 40%. Finally, the patient was diagnosed with IgG4-TIN complicated with RPF and given glucocorticoids as long-term maintenance therapy, helping him keep out of dialysis. After a follow-up of 19 mo, the patient had recovered well. Previous literature on IgG4-RKD and RPF was retrieved from PubMed to characterize the clinical and pathological features and to identify the diagnosis and treatment of IgG4-RKD.
CONCLUSION
Our case report demonstrates the clinical characteristics of IgG4-RKD complicated with RPF. Serum IgG4 is a favorable indicator for screening. Performing renal biopsy actively plays a vital role in diagnosis and treatment, even if the patient has a long course and manifests with renal insufficiency. It is remarkable to treat IgG4-RKD with glucocorticoids. Hence, early diagnosis and targeted therapy are essential for reversing renal function and improving extrarenal manifestations in patients with IgG4-RKD.
Core Tip: IgG4-related kidney disease is a rare disease with unknown etiology. The disease has various clinical manifestations because it often combines with damage to other organs, which leads to easy missed diagnosis or misdiagnosis. Presently, the diagnosis is mainly dependent on kidney biopsy, and the main treatment is glucocorticoids. Our case report demonstrates the clinical manifestations of IgG4-related tubulointerstitial nephritis complicated with retroperitoneal fibrosis. After diagnosis by renal biopsy, the patient was treated with glucocorticoids and finally removed from dialysis with a favorable outcome.
