Zhang J, Wang X, Zou GM, Li JY, Li WG. Membranous nephropathy with systemic light-chain amyloidosis of remission after rituximab therapy: A case report. World J Clin Cases 2023; 11(23): 5538-5546 [PMID: 37637680 DOI: 10.12998/wjcc.v11.i23.5538]
Corresponding Author of This Article
Wen-Ge Li, MD, PhD, Professor, Department of Nephrology, China-Japan Friendship Hospital, No. 2 Yinghuayuan East Street, Beijing 100029, China. wenge_lee2002@126.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/
World J Clin Cases. Aug 16, 2023; 11(23): 5538-5546 Published online Aug 16, 2023. doi: 10.12998/wjcc.v11.i23.5538
Membranous nephropathy with systemic light-chain amyloidosis of remission after rituximab therapy: A case report
Jiao Zhang, Xu Wang, Gu-Ming Zou, Jia-Yi Li, Wen-Ge Li
Jiao Zhang, Beijing University of Chinese Medicine, Beijing 100029, China
Xu Wang, Gu-Ming Zou, Jia-Yi Li, Wen-Ge Li, Department of Nephrology, China-Japan Friendship Hospital, Beijing 100029, China
Author contributions: Zhang J contributed to the collection of data, drafting and revising of the manuscript; Wang X contributed to the follow-up of the patient; Li JY and Zou GM provided pathological assistance; Li WG contributed to the treatment of the patient and the revision of the manuscript; All authors read and approved the final manuscript.
Informed consent statement: The study has been approved by the ethics committee of the China-Japan Friendship Hospital. Written informed consent was obtained from the individual for the publication of any potentially identifiable images or data included in this article.
Conflict-of-interest statement: All the authors have no conflicts of interest to declare.
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: Wen-Ge Li, MD, PhD, Professor, Department of Nephrology, China-Japan Friendship Hospital, No. 2 Yinghuayuan East Street, Beijing 100029, China. wenge_lee2002@126.com
Received: March 31, 2023 Peer-review started: March 31, 2023 First decision: July 3, 2023 Revised: July 17, 2023 Accepted: July 25, 2023 Article in press: July 25, 2023 Published online: August 16, 2023
Abstract
BACKGROUND
About 70%-80% of patients with primary membranous nephropathy (MN) have phospholipase A2 receptor (PLA2R) in renal tissue. Systemic light-chain (AL) amyloidosis is the most common type of amyloidosis. MN complicated with amyloidosis is rare.
CASE SUMMARY
A 48-year-old Chinese male presented with nephrotic syndrome, positive serum PLA2R antibody, and positive serum and urine IgG-lambda type M-protein, with a normal ratio of serum-free light-chain level. The patient was diagnosed with MN accompanied by AL amyloidosis. He was treated with rituximab with glucocorticoids and CyBorD regimen of chemotherapy. After 21 mo of follow-up, the patient achieved complete remission regarding nephrotic syndrome without adverse effects of chemotherapy.
CONCLUSION
We report a case of PLA2R-related MN complicated with primary AL amyloidosis only with renal involvement and successfully treated with rituximab, glucocorticoids and chemotherapy.
Core Tip: We report a rare case of phospholipase A2 receptor-related membranous nephropathy complicated with primary systemic light-chain amyloidosis with only renal involvement. The patient achieved complete remission after receiving rituximab, glucocorticoids and CyBorD regimen chemotherapy. Additionally, we conducted a literature review to provide current evidence on early diagnosis and treatment of this disease.
