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Abstract: FR-PO621

The Great Clinical Masquerade of ANCA Vasculitis

Session Information

Category: Glomerular Diseases

  • 1302 Glomerular Diseases: Immunology and Inflammation

Authors

  • Singh, Aditi, UConn Health, Farmington, Connecticut, United States
  • Simeone, Stephen N., UConn Health, Farmington, Connecticut, United States
  • Jweehan, Duha A., UConn Health, Farmington, Connecticut, United States
  • Shah, Mamta, UConn Health, Farmington, Connecticut, United States
Introduction

Early recognition and treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (GN) is paramount to halt progression to end stage renal disease. We present a case of new-onset ANCA positive renal-limited vasculitis in an elderly female with failure to thrive.

Case Description

A 78-year-old female presented to our institution with altered mental status and poor oral intake. Over the past several months, she had progressive clinical decline with recurrent admissions for suspected interstitial pneumonia, urinary tract infections (UTI) and dysphagia with failure to thrive. Chemistry demonstrated a serum creatinine of 2.5 mg/dL elevated from a baseline of 0.8 mg/dL.The patient received intravenous crystalloid resuscitation and antimicrobial therapy for clinical hypovolemia and suspicion of UTI. Urinalysis revealed large proteinuria with a urine protein-creatinine ratio of 3.35, hematuria, pyuria, and fine granular casts. Further evaluation revealed positive speckled antinuclear antibodies with titers of 1:160, positive perinuclear-ANCA with a titer of 1:1280 and positive myeloperoxidase antibodies. Renal biopsy demonstrated active crescentic necrotizing glomerulonephritis (Figure 1), significant interstitial inflammation with vascular involvement and negative immunofluorescence. High dose corticosteroid and cyclophosphamide therapy was initiated with rapid improvement in her renal function and resolution of hematuria. She clinically improved and was discharged on prednisone and cyclophosphamide with a plan to transition to rituximab.

Discussion

We present a case that highlights the diagnostic challenge of ANCA-associated vasculitis with its protean symptoms and multisystem involvement in an aging population where multiple comorbidities co-exist. ANCA positive GN is the most common new onset GN in adults over the age of 50 and yet studies show a significant delay between initial presentation and diagnosis. A high degree of clinical suspicion is required in this challenging population to reduce the morbidity and mortality associated with this disease.

Circumferential cellular crescent