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Abstract: TH-PO679

Avacopan and ANCA

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Hussaini, Sadia Syed, University of Rochester Medical Center, Rochester, New York, United States
  • Sabescumar, Janany Jansy, University of Rochester Medical Center, Rochester, New York, United States
Introduction

ANCA-negative vasculitis, although rare, is associated with increased morbidity and mortality. In this case report, we present a patient with ANCA-negative vasculitis and highlight the use of the novel agent Avocopan.

Case Description

A seventy-four-year-old male with a medical history of hypertension, insulin-dependent diabetes, and coronary arterial disease presented to urgent care with symptoms of shortness of breath, cough, lower extremity swelling, and a rash on his buttocks. He was thought to have multifocal pneumonia and heart failure. Despite initial treatment, his edema worsened, and subsequent laboratory tests showed an increase in serum creatinine from 1.7 mg/dL to 3.75 mg/dL. Urinalysis revealed microscopic hematuria and proteinuria quantified at 8.89 g/day. Concerns arose regarding rapidly progressive glomerulonephritis, and a secondary serological workup yielded largely negative results, including ANCA. A kidney biopsy was performed, revealing pauci-immune crescentic glomerulonephritis of the focal necrotizing type with moderate activity and minimal chronicity. The patient underwent Rituximab induction therapy along with steroids for maintenance. However, due to worsening edema, hypertension, hyperglycemia, and weight gain a decision was made to switch the patient to Avacopan. At six months follow up, the patient's serum creatinine reduced to 1.89 mg/dL, and proteinuria decreased to 0.81 g.

Discussion

The standard approach to ANCA-mediated vasculitis has involved the use of glucocorticoids in combination with Rituximab or cyclophosphamide. However, a novel agent called Avacopan has emerged as a promising alternative. Avacopan specifically targets the alternative complement pathway, which is believed to play a role in the pathogenesis of ANCA vasculitis. In our case of ANCA-negative vasculitis the patient responded well to Avacopan therapy, achieving remission with minimal proteinuria. References: Jayne et Al. Avacopan for the Treatment of ANCA-Associated Vasculitis.NEJM 2021 Feb 18 384:599-609. Jennette JC, Nachman PH. ANCA Glomerulonephritis and Vasculitis. Clin J Am Soc Nephrol. 2017 Oct 6;12(10):1680-1691.