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Abstract: PO1963

Minimal Change Disease in Systemic Lupus Erythematosus: An Infrequent Variant

Session Information

Category: Trainee Case Report

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Naeem, Ehsun, Beaumont Health, Royal Oak, Michigan, United States
  • Kellerman, Paul S., Beaumont Health, Royal Oak, Michigan, United States
  • Mansuri, Saima, Beaumont Health, Royal Oak, Michigan, United States
Introduction

Lupus Nephritis (LN) is thought to complicate the disease course of almost half of all patients diagnosed with Systemic Lupus Erythematosus (SLE). While nephrotic syndrome (NS) in these patients is usually due to type IV/V Lupus Nephritis (LN), it may in rare instances occur secondary to minimal change disease (MCD), a phenomenon known as Lupus Podocytopathy (LP). We report a case of a young female with LP with concomitant Acute Tubular Necrosis (ATN)

Case Description

40 year old female, known case of SLE (not on maintenance immunosuppression) and Hypertension presented with bright red blood per rectum and dyspnea for 2 weeks. Review of systems was pertinent for generalized swelling and facial rash. On initial assessment, she was hypertensive and physical exam revealed facial swelling, discoid rashes and 2+ lower Extremity edema bilaterally. Workup revealed Normocytic Anemia, Acute Kidney injury, Hyperkalemia and Metabolic Acidosis. Urine studies showed nephrotic-range proteinuria and hematuria but were negative for casts. Free K/L ratio was high at 2.32 and C3 levels low at 42 mg/dL. Ultrasound guided kidney biopsy showed mild thickening of GBM and dilated tubules with diminished brush borders in the absence of crescentic changes. Electron Microscopy noted diffuse fusion of foot processes, along with rare intramembranous deposits. Immunofluorescence revealed a full house staining pattern within the Mesangium and the patient was diagnosed with Lupus Podocytopathy with concurrent LN Type I. Substantial reduction in proteinuria was noted with a brief course of Prednisone

Discussion

Our patient with SLE presented with NS and AKI, features typical of membranous/proliferative LN. Interestingly, her biopsy findings provided little evidence of endocapillary proliferation or sub-epithelial IC deposits and were more consistent with MCD, suggestive of Lupus podocytopathy. LP is rare to the extent that it does not form part of the official WHO classification for LN and has only been described a handful of times in prior literature, mostly in the form of case reports. However, given its prognostic implications, LP remains an important consideration in the evaluation of NS in SLE patients. While patients with Type IV/V LN require aggressive immunosuppressive therapy, patients with LP frequently respond well to steroids alone and have a much slower progression of disease