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Kidney Week

Abstract: SA-PO0796

Membranous Nephropathy, Phospholipase A2 Receptor (PLA2R)-Antibody Positivity, and Monoclonal Deposits in a Patient with HIV

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Shrestha, Rajesh, University of Maryland Medical Center, Baltimore, Maryland, United States
  • Agarwal, Neil Kumar, University of Maryland Medical Center, Baltimore, Maryland, United States
  • Chandra, Preeti, University of Maryland Medical Center, Baltimore, Maryland, United States
Introduction

Glomerular disease in patients with HIV. on antiretroviral treatment (ART), includes HIV associated immune complex mediated GN (HIVICK), that can manifest as Membranous nephropathy (MN). The immune deposition in PLA2R MN is polytypic. We described a rare case PLA2R antibody positive MN in a patient with HIV with light chain restriction on renal biopsy.

Case Description

The patient is a 35-year-old Black male, with HIV, on ART, presenting with edema, hypoalbuminemia, proteinuria. CD4 count was 438 cells/microL. Viral load (VL) was low/undetectable. On presentation., creatinine 0.7-0.9 mg/dL range, urine protein creatinine ratio (UPCR) was 2 g/g, serum albumin 2.7 g/dL. Subsequently UPCR was up to 7 g/g. Renal biopsy showed membranous nephropathy with lambda light chain restriction. Light microscopy: mild mesangial hypercellularity, C4d stain positive in membranous pattern, minimal tubular atrophy, interstitial fibrosis, negative Congo red for amyloid. IF: 3+ granular membranous staining for lambda light chain, negative for IgG, IgM, IgA, C3, C1q, kappa light chains. Immunostaining for IgG4 was positive in membranous pattern. EM: large subepithelial electron-dense deposits, extensive podocyte foot process effacement. Mass spectrometry, laser dissection showed peptide profile consistent with PLA2R. PLA2R ab was elevated to 181 RU/mL. ANA, C3, C4, Hepatitis B, C, RPR were negative. Serum protein electrophoresis (SPEP) showed trace IgG kappa and trace lambda light chain band. Urine protein electrophoresis was negative.
Bone marrow (BM) biopsy was unremarkable. PLA2R ab has risen to ~486 RU/mL, creatinine to 1.5 mg/dL, serum albumin is in 1.9-2.2 range. Treatment with Rituximab has been initiated.

Discussion

MN is associated with polytypic antibodies/deposits, most commonly against PLA2R antigen. MN with monotypic deposits and light chain restriction is rare, occurring in about 0.95% of MN cases. The combination of PLA2 R positive MN that is monotypic is rare. MN has been documented in patients with HIV, mostly occurring with low/undetectable VL, about half of such patients are PLA2R ab positive. The combination of PLA2R ab positive MN with light chain restriction in a patient with HIV has to our knowledge not been described before. The PLA2R ab positive MN with light chain restriction, positive biclonal band on SPEP, likely reflects immune dysregulation triggered by HIV in this patient.

Acknowledgment

Dr. Hector Mesa-Corrales, Professor of Pathology, Department of Pathology, University of Maryland Medical Center for providing the histopathology slides.