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Abstract: SA-PO1015

A Case of Steroid Refractory Lupus Podocytopathy: Is Glucocorticoid Monotherapy Sufficient?

Session Information

Category: Glomerular Diseases

  • 1403 Podocyte Biology

Authors

  • Lawson, Cameron T., East Carolina University, Greenville, North Carolina, United States
  • Portz, Brent J., Eastern Nephrology Associates PLLC, Greenville, North Carolina, United States
Introduction

Lupus Podocytopathy (LP) is a rare, but clinically impactful, manifestation of Systemic Lupus Erythematous (SLE). Accepted management of LP, Minimal Change (MC) and FSGS - subtypes alike, utilizes glucocorticoid (GC) monotherapy for proteinuria suppression. We present a case of high-grade podocyte effacement LP with MC subtype refractory to conventional GC management requiring the addition of an immunosuppressive agent.

Case Description

38 yo female with SLE and no chronic kidney disease presented with nephrotic syndrome and acute kidney injury. Creatine on presentation was 5.7mg/dL and urine protein:creatinine ratio 11g. Serologies demontrated +ANA > 1:640 and +dsDNA. Renal biopsy was performed and suggestive of LP - MC subtype. Electron Microscopy: diffuse (90%) visceral epithelial foot process effacement; no endothelial tubuloreticular inclusions or subendothelial immunocomplex deposits were noted (Figure 1). Light Microscopy and immunofluorescence were unremarkable for chronicity or proliferative nephritis. High-dose prednisone was initiated at 1mg/kg. No significant change in nephrotic syndrome was observed despite 4 months of prednisone monotherapy. Mycophenolate Mofetil (MMF) was subsequently incorporated achieving complete remission of proteinuria and return to baseline creatinine.

Discussion

Small retrospective studies evaluating LP have suggested GC monotherapy should be successful in achieving clinical remission, especially in MC subtypes. The case presented suggests the addition of a non-glucocorticoid agent may be required to mitigate the proposed pathogenic cytokine and T-cell dysfunctions. Factors such as high-grade podocyte effacement and heavy proteinuria should prompt consideration for empiric dual-agent immunosuppression. Larger-scale randomized studies are needed to demonstrate the clinical and pathologic criteria determining single vs. dual immunosuppressive therapy for LP.

Figure 1 Electron Microscopy