Abstract: FR-PO0737
Rapid Progression to ESKD in a Patient with Tissue-Positive, Seronegative Phospholipase A2 Receptor Membranous Nephropathy
Session Information
- Glomerular Diseases: Membranous Nephropathy, FSGS, and Podocytopathies
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Khodor, Hawaa, LSU Health Shreveport, Shreveport, Louisiana, United States
- Ahmed, Mohamed Mohyedin, LSU Health Shreveport, Shreveport, Louisiana, United States
- Zubair, Maha, LSU Health Shreveport, Shreveport, Louisiana, United States
- Morisetti, Phani Purushotham, LSU Health Shreveport, Shreveport, Louisiana, United States
Introduction
Membranous nephropathy (MN) is a leading cause of nephrotic syndrome in adults and is commonly associated with circulating antibodies against the phospholipase A2 receptor (PLA2R). However, discordance between serum antibody testing and tissue PLA2R expression is increasingly recognized and may complicate diagnosis
Case Description
A 55-year-old African American woman with hypertension and type 2 diabetes mellitus presented with nephrotic syndrome and rapidly progressive kidney failure. Over three months, serum creatinine increased from 1.8 mg/dL to 16.6 mg/dL. Urine protein-creatinine ratio was 7.8 g/g. Evaluation for secondary causes was negative, including hepatitis B/C, HIV, ANA, ANCA, complement levels, serum PLA2R and THSD7A antibodies, serum protein electrophoresis, and free light chains. Renal ultrasound showed normal-sized kidneys without chronic structural abnormalities.
Kidney biopsy demonstrated PLA2R-positive membranous nephropathy with stage II subepithelial deposits on electron microscopy. Light microscopy revealed advanced chronic injury, including global glomerulosclerosis in 9/14 glomeruli, focal segmental glomerulosclerosis, moderate interstitial fibrosis/tubular atrophy, and severe arteriosclerosis with arteriolar hyalinosis. Immunofluorescence showed granular capillary wall IgG (2+) staining with positive PLA2R staining. Due to progressive renal failure, the patient was started on hemodialysis and remained dialysis-dependent at follow-up.
Discussion
This case highlights an important discordance between negative circulating PLA2R antibodies and positive tissue PLA2R expression in MN. Despite seronegativity, biopsy confirmed PLA2R-associated disease, emphasizing the essential role of kidney biopsy in suspected MN. The patient’s unusually rapid progression to end-stage kidney disease likely reflected advanced chronic histologic injury, vascular disease, superimposed segmental sclerosis, and possible genetic susceptibility given a family history of ESKD. This case underscores the importance of tissue PLA2R staining in patients with rapidly progressive nephrotic syndrome despite negative serologic testing.