Abstract: PUB400
Avacopan as Adjuvant Therapy for ANCA-Associated Vasculitis: A Case Report
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Gupta, Sanjeev, Westchester Medical Center Health Network, Valhalla, New York, United States
- Papanagnou, Anastasios, NewYork-Presbyterian Hudson Valley Hospital, Cortlandt Manor, New York, United States
- Mittal, Amol, Westchester Medical Center Health Network, Valhalla, New York, United States
- Chugh, Savneek S., Westchester Medical Center Health Network, Valhalla, New York, United States
Introduction
The anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis are severe systemic disorders marked by small-vessel vasculitis. This involve autoantibodies against neutrophil proteins, particularly leukocyte proteinase 3 (PR3) or myeloperoxidase (MPO). Standard treatment for ANCA vasculitis often includes high-dose steroids, rituximab, or cyclophosphamide. Rituximab is the preferred option, but patients who do not respond sufficiently might be switched to cyclophosphamide. However, cyclophosphamide's significant side effects necessitate its limited use. We present a case of partial response to rituximab, who, instead of increasing steroids or using cyclophosphamide, was treated with avacopan, resulting in a favorable outcome.
Case Description
A 48-year-old male with hyperlipidemia presented with rash on palms and soles, and shortness of breath. Initial workup showed hemoglobin of 11.8 g/dl, platelet count of 437k/mm, BUN of 16mg/dl, and creatinine of 1.34mg/dl. Urinalysis indicated 3+ protein and 136 RBCs. Further tests revealed a urine protein-to-creatinine ratio of 1.3, negative hepatitis panel, negative ANA, and normal C3 and C4. Anti-MPO antibodies were 109 units, anti-PR3 antibodies were 2.35 units,and anti-GBM ab was negative. Kidney biopsy confirmed ANCA-associated renal disease with focal glomerular fibrinoid necrosis and crescents. The patient received 60 mg prednisone and 1 g Rituximab. He returned with worsening creatinine and shortness of breath within a week, was diagnosed with cardiac tamponade, and underwent a pericardial window. Despite moderate increased prednisone, his creatinine continued to rise with active sediments in urine. Avacopan was initiated. Within a week, his creatinine improved to baseline, and urine analysis normalized. He completed 4 rituximab doses and 52 weeks of avacopan. Eighteen months later, his creatinine remains stable at 1.3mg/dl.
Discussion
Avacopan, used as an induction therapy alongside rituximab and steroids, provides an alternative to high-dose steroids, typically tapered within a month to reduce toxicity. Despite the lack of case reports on avacopan as adjuvant therapy for partial responders to standard ANCA vasculitis treatment, our patient's positive outcome highlights its potential benefit. To date, the patient's kidney function remains stable, emphasizing avacopan's role in vasculitis management