Membranous nephropathy with systemic light-chain amyloidosis of remission after rituximab therapy: A case report

doi:10.12998/wjcc.v11.i23.5538

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World Journal of Clinical Cases

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Case Report

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).

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
Processing time: 137 Days and 16.9 Hours

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.

Keywords: Amyloidosis; Membranous nephropathy; Phospholipase A2 receptors; Rituximab; Renal biopsy; Case report

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.