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Kidney Week

Abstract: SA-PO0778

Fulminant Seronegative Lupus Nephritis and the Challenge of Kidney Biopsy

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Gomez Montoya, Luisa Fernanda, Prime South GME Consortium- Knapp Medical Center, Weslaco, Texas, United States
  • Simono, Krystal, Prime South GME Consortium- Knapp Medical Center, Weslaco, Texas, United States
  • Jagarlamudi, Nikhil Sai, Prime South GME Consortium- Knapp Medical Center, Weslaco, Texas, United States
  • Nelakuditi, Bhavana, Prime South GME Consortium- Knapp Medical Center, Weslaco, Texas, United States
Introduction

Lupus Nephritis typically presents in association with positive autoimmune serologies and classic mucocutaneous manifestations of Systemic Lupus Erythematosus. Seronegative Lupus Nephritis is an uncommon and diagnostically challenging entity, especially in patients with comorbidities.

Case Description

We report the case of a 24-year-old female with chronic kidney disease, congenital AV block status post pacemaker placement, chronic pancytopenia, and morbid obesity presenting with 4 weeks of progressive painful anasarca, dyspnea, dry cough and dizziness. Laboratory evaluation showed pancytopenia, creatinine of 2.6 mg/dL, nephrotic-range proteinuria of 11–13 g/day and 3+ hematuria. Extensive autoimmune workup, including ANA, anti-dsDNA, anti-Smith and SSA/SSB antibodies was negative, with normal complement levels. Despite significant proteinuria and renal dysfunction, renal biopsy was initially deferred due to high procedural risk in the setting of her comorbidities. Negative serologies complicated the diagnostic evaluation, making renal biopsy necessary for diagnosis. Kidney biopsy ultimately confirmed Class IV proliferative and Class V membranous lupus nephritis.
Her hospitalization was complicated by a post-biopsy subcapsular renal hematoma, cardiogenic shock, necrotizing mucocutaneous lesions, vasculitic purpura and microangiopathic hemolysis. Despite treatment with high-dose corticosteroids and mycophenolate mofetil, her renal and hemodynamic status worsened, requiring transfer to a tertiary care center for plasmapheresis and rituximab therapy.

Discussion

Negative autoimmune workup may obscure and delay the diagnosis of fulminant lupus nephritis and underlying SLE. Renal biopsy plays a pivotal role in establishing the diagnosis and guiding treatment. This case highlights the challenging balance between procedural risks of renal biopsy in medically complex patients and consequences of delayed recognition of aggressive lupus nephritis. Negative serology should not exclude lupus nephritis when clinical suspicion for immune-mediated glomerular disease is high.