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Kidney Week

Abstract: PO1959

A Rare Case of ANCA-Negative Pauci-Immune Crescentic Glomerulonephritis in an Elderly Woman

Session Information

Category: Trainee Case Report

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Garcia Valencia, Oscar Alejandro, University of Miami /Jackson Memorial Hospital, Miami, Florida, United States
  • Edelman Saul, Eduardo, University of Miami /Jackson Memorial Hospital, Miami, Florida, United States
  • Olivera Arencibia, Yanetsy, University of Miami /Jackson Memorial Hospital, Miami, Florida, United States
  • Sosa, Marie A., University of Miami /Jackson Memorial Hospital, Miami, Florida, United States
Introduction

Acute kidney injury is usually multifactorial with a broad differential diagnosis. Of those, rapidly progressive glomerulonephritis (RPGN) requires special attention as it represents a true diagnostic and therapeutic emergency that can lead to irreversible kidney failure.

Case Description

A 92-year-old female with a history of Chronic Kidney Disease Stage III, Diabetes Mellitus type II, hypertension, and recurrent deep vein thrombosis sent to the ER for evaluation of rapidly worsening kidney function found on outpatient laboratories for assessment of poor oral intake and fatigue for 2 weeks. On arrival, BP was 133/49mmHg, HR 70bpm, temperature 36.9°C, RR 17 rpm and SO2 of 99%. Mental status was at baseline and physical exam was unremarkable. Laboratory data were remarkable for serum creatinine of 10.55 mg/dl from baseline of 1.8 mg/dl, potassium level of 6 mEq/L and bicarbonate of 17 mEq/L. Urinalysis showed proteinuria (30+), hematuria (344 RBCs/hpf) and leukocyturia (68 WBC/hpf). Urine protein creatinine ratio of 2.4. No acute EKG changes. Renal ultrasound demonstrated increased bilateral echogenicity; otherwise unremarkable. Patient initially treated medically with no improvement requiring hemodialysis. Work up sent with ANCA panel, Anti-GBM, Serum protein electrophoresis, immunofixation, Free Light Chains, complement and Hepatitis panel all negative. Acutely worsening kidney function coupled with active sediment and proteinuria prompted a kidney biopsy which demonstrated pauci-immune crescentic GN. She was started on Methylprednisolone 500mg IV daily for 3 days, followed by prednisone 60mg qd and plasmapheresis every-other-day.

Discussion

PICG is a potentially life-threatening condition leading to renal failure within days or weeks and represents up to 80% of RPGN. The majority of cases (~90%) are ANCA positive and occur in younger patients. Here we present the clinical profile of a rare new diagnosis of ANCA negative PICG in an elderly lady. Renal damage is more severe and kidney survival poorer when compared to ANCA positive crescentic GN. Our patient
received RRT throughout hospital stay showing signs of recovery on second week. She was discharged on oral Prednisone 30mg qd and Cyclophosphamide 50mg qd with close follow up.