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;
11:5538-5546. [PMID:
37637680 PMCID:
PMC10450367 DOI:
10.12998/wjcc.v11.i23.5538]
[Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/31/2023] [Revised: 07/17/2023] [Accepted: 07/25/2023] [Indexed: 08/16/2023] Open
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.
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