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Kidney Week

Abstract: FR-PO0740

An Unusual Presentation of Membranous Nephropathy with Primary and Secondary Features

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Shah, Aanya Dar Mudita, James Martin High School, Arlington, Texas, United States
  • Baker, Stephen, Baylor Scott & White Health, Fort Worth, Texas, United States
  • Shah, Dar B., Lone Star Kidney Care, P.L.L.C., Fort Worth, Texas, United States
Introduction

Membranous nephropathy (MN) is a syndrome commonly seen in adults and is a cause of nephrotic syndrome. Membranous nephropathy is a group of diseases which share a common histopathological pattern including the presence of immunoglobulin and complement-containing immune deposits in a subepithelial pattern.1 In 2009, antibodies against the M-type PLA2R were identified in an adult with idiopathic MN.

Case Description

In 2013, a 75-year-old female presented to our clinic with lower extremity swelling. In summary, we had a patient with proteinuria and hematuria. A CT guided kidney biopsy in order to establish a definitive diagnosis was done.
Despite showing evidence for diffuse immunoglobulin staining, the patient was diagnosed with primary membranous nephropathy.
The patient was started on immunosuppression with a combination of prednisone and cyclosporine. The patient had remission of disease.
In 2024, the patient returned to the office complaining of recurrent lower extremity swelling. Despite having biopsy features once again of secondary, more specifically Class V lupus nephropathy, diagnosed with primary membranous nephropathy due to the positive anti PLA2R staining. She was started on treatment with Mycophenolate mofetil. She responded favorably achieved remission of proteinuria, which persists.

Discussion

This case is an example of an unusual presentation of membranous nephropathy with both primary and secondary features. While staining for anti PLA2R has been described in lupus Class V membranous nephropathy, it is rare.
In our patient, response to Cyclosporine during the first course of treatment was excellent. At relapse, she was treated like a patient with class V lupus membranous nephropathy rather than primary nephropathy despite PLA2R levels being positive. She had an excellent response to treatment, except this time the drug of choice was Mycophenolate Mofetil. In both instances, the patient was also treated with Prednisone.This case is a unique example of a kidney biopsy with features that suggest lupus membranous nephropathy, but which stains positively for PLA2R antibodies.