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Published on: August 23, 2024
Membranous nephropathy with light chain restricted deposits
Raja Ramachandran1, Neeraj Inamdar1, Joyita Bharati1
1Department of Nephrology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
Membranous nephropathy (MN) with monoclonal deposits is rare. Conventional therapies show efficacy in non-malignant cases, with some patients achieving remission.
Area of Science:
- Nephrology
- Immunopathology
- Internal Medicine
Background:
- Membranous nephropathy (MN) with monoclonal immunoglobulin deposits on immunofluorescence (IF) is infrequently documented.
- Limited data exists on the clinical course and management outcomes for this specific MN subtype.
Purpose of the Study:
- To report the clinical experience and management outcomes of five patients diagnosed with MN and monoclonal deposits.
- To evaluate the efficacy of conventional therapies in this rare condition.
Main Methods:
- Retrospective case series of five patients with MN and monoclonal deposits.
- Analysis of clinical data, including proteinuria, serum albumin, serum creatinine, and immunofluorescence findings.
- Assessment of treatment responses to therapies such as cyclophosphamide, steroids, Rituximab, and Bortezomib/Thalidomide/Dexamethasone.
Main Results:
- Four patients were phospholipase A2 receptor (PLA2R) negative, and one was PLA2R positive.
- Monoclonal deposits included IgG3/k, IgG3/λ, and IgG3/IgG4k subtypes.
- Two patients treated with cyclophosphamide and steroids achieved complete remission; one showed reduced creatinine with persistent proteinuria.
- One PLA2R-positive patient treated with Rituximab achieved complete remission.
- A patient with elevated free light chains achieved remission but later experienced renal function decline.
Conclusions:
- Conventional therapies appear effective for non-malignant MN with monoclonal deposits.
- Further research is needed due to the limited number of cases presented.
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