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Abstract: PO1962

A Case of Lupus Podocytopathy (LP) with Focal Segmental Glomerulosclerosis (FSGS): Is It Time to Add LP to the Next Revision of the Classification of Lupus Nephritis?

Session Information

Category: Trainee Case Report

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Zahid, Hasan, University of Florida Health, Gainesville, Florida, United States
  • Carlson, Jeremy, University of Florida Health, Gainesville, Florida, United States
  • Hamdani, Muhammad usama shah, University of Florida Health, Gainesville, Florida, United States
  • Shah, Chintan Vimalkumar, University of Florida Health, Gainesville, Florida, United States
Introduction

Lupus podocytopathy is not included in the commonly used International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification of Lupus Nephritis (LN). It has been reported in literature for the last 20 years. LP can have pathologic transition with variable outcomes. We describe a case of LP with FSGS in a young male patient with subsequent relapse.

Case Description

25 year old male with past history of lupus without nephritis and chronic immune mediated thrombocytopenia presented with generalized fatigue. Physical exam revealed diffuse rash and vital signs were within normal limits. Relevant laboratory findings included platelet count of 21000/microliter, acute kidney injury with creatinine (Cr) of 1.6 mg/dl (baseline Cr of 0.8 to 1.1 mg/dl), spot urine protein creatinine ratio of 4.3 g, 24 hour urine protein of 4.1 g, low C3 and C4, hemoglobin of 9.6 g/dl and WBC count of 3.6/microliter. Serology was positive for ANA, dsDNA and SSA indicating active lupus flare. Left kidney biopsy showed mild mesangial expansion, no endocapillary proliferation and subtotal (>80%) podocyte foot process effacement. No subendothelial or subepithelial deposits were seen. He was treated with pulse dose steroids followed by oral steroids and serum Cr came back to baseline. Subsequently he was treated with mycophenolate mofetil 2 g/day. At 2 weeks, proteinuria came down to 1.8 g/day and by 12 weeks he achieved remission. However at 6 months, he had urine protein of 8.8 g in 24 hours with increase in Cr to 2.4 from 1.6, suggesting relapse.

Discussion

The presence of minimal or no capillary wall immune deposits with or without mesangial proliferation and effacement of podocyte foot processes in the setting of nephrotic range proteinuria is collectively termed as LP. LP must be considered in patients with lupus presenting with NS (Nephrotic Syndrome). It is cardinal that we consider adding this distinct entity in the classification of lupus nephritis.