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Abstract: SA-PO867

Extreme Fatigue as the Only Complaint in Double-Positive Anti-Glomerular Basement Membrane (GBM) and ANCA Disease: An Unusual Presentation

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: From Inflammation to Fibrosis

Authors

  • Cintrón-García, Juan J., Loyola University Health System, Maywood, Illinois, United States
  • Vellanki, Kavitha, Loyola University Health System, Maywood, Illinois, United States
  • Picken, Maria M., Loyola University Health System, Maywood, Illinois, United States
  • Zghayer, Aseel, Loyola University Health System, Maywood, Illinois, United States
Introduction

Acute glomerulonephritis (GN) due to anti-glomerular basement membrane (GBM) disease is extremely rare with reported incidence of one per million. Double positive disease with anti-GBM phenotype on kidney biopsy and ANCA positive titers on serology is reported to present in older population with high relapse rates compared to anti-GBM disease alone. Extreme fatigue as the only presenting complaint is uncommon, here we present one such case.

Case Description

76 year old female who leads an active life-style presents to her PCP's office with extreme fatigue of 3 weeks duration. Initial work-up revealed AKI with serum creatinine of 4.19 mg/dL. She was admitted for further evaluation. Urinalysis was +ve for large blood, >10 RBC/HPF, and protein. She received IV fluids, serology for GN work up was sent and discharged home. She was re-admitted later as myeloperoxidase (MPO)-ANCA titers were reported positive. Kidney biopsy revealed cresenteric GN with 85% glomerular involvement. Treatment was initiated with pulse steroids, rituximab and plasma exchange for ANCA vasculitis till kidney biopsy findings confirmed anti GBM disease (Figure 1), anti-GBM titer results following a few days after kidney biopsy. Response to treatment is shown in Figure 2.

Discussion

Double-positive vasculitis with anti-GBM and ANCA is rare. Our patient did not have any lung involvement. While Rituximab alone for induction is not commonly reported in double positive disease, our patient was extremely frail and hence did not receive cyclophosphamide. A high degree of clinical suspicion for acute GN is needed when elderly patients present with AKI and an abnormal UA, extreme fatigue could be the only complaint.

Figure 1

Figure 2