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Kidney Week

Abstract: FR-PO714

A Case of Lupus-Like Nephritis in a Patient with a Negative Antinuclear Antibody (ANA)

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: From Inflammation to Fibrosis

Authors

  • Elhilali, Osama, University of Florida College of Medicine - Jacksonville, Jacksonville, Florida, United States
  • Rashid, Tasnuva, University of Florida College of Medicine - Jacksonville, Jacksonville, Florida, United States
  • Liu, Shiguang, University of Florida College of Medicine - Jacksonville, Jacksonville, Florida, United States
  • Hasan, Irtiza, University of Florida College of Medicine - Jacksonville, Jacksonville, Florida, United States
Introduction

Diagnosis of SLE cannot be made based only on kidney biopsy, however the histopathologic findings is one of the important cornerstone for the diagnosis of lupus nephritis.

Case Description

A 60-year-old male with PMH of HTN presented with worsening bilateral lower extremity edema with hematuria & heavy proteinuria for 4 months. Other symptoms significant of dyspnea on exertion & a strong family history of kidney disease. Labs showed S. Cr 1.80 mg/dL (previous 0.86) & urine protein/creatinine >7.96 g/g. Workups including ANA, ANCA, C3 (160), C4 (36), and ds-DNA (<1) were negative. Serum protein electrophoresis revealed two small oligoclonal bands without monoclonal paraprotein. CXR, TTE & renal ultrasound were within normal limit. HBsAg, anti-HCV, RPR & HIV were also negtive. A kidney biopsy revealed marked (>50%) endocapillary proliferation, mesangial sclerosis, GBM thickening with two cellular crescents. Immunofluorescence (IF) studies showed full house positivity on immunoglobulins (Ig A, Ig G and Ig M) & complement deposit. Electron microscopy (EM) showed subendothelial & mesangial electron dense depositions with no tubuloreticular inclusion identified. Patient was initially treated with IV methylprednisolone followed by prednisone with improvement in renal function. Patient was discharged on mycophenolate for outpatient follow up and monitoring.

Discussion

Here we presented a case of renal-limited, lupus-like nephritis in a patient with absent extrarenal manifestations & negative lupus serology. As per American Rheumatism Association (ARA) criteria, our patient did not fit into the diagnosis of SLE. However, the presence of immune-complex mediated glomerulonephritis and “full house” IF staining and EM showing subendothelial and mesangial electron dense deposits on kidney biopsy are highly suggestive of the lupus nephritis. There is a need for proper characterization of this disease pathogenesis, precise diagnostic criteria, standardization of treatment protocols & long term follow up studies to identify the true prognosis of this challenging clinical entity.

Histopathologic images of a kidney biopsy