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Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a rare condition that can cause rapid renal failure. Treatment involves steroids and other immunosuppressive agents. Agents for induction include rituximab, cyclophosphamide, pulse dose steroids and avacopan.
Maintenance regimens include tapered doses of steroids, azathioprine and rituximab
We present a case of severe AAV that maintained remission with a protracted course of low dose prednisone without maintenance rituximab or azathioprine.
A 70-year-old woman was admitted for acute kidney injury (AKI), with a serum creatinine (sCr) of 6.93 mg/dL (baseline sCr of 0.9 mg/dL, nil proteinuria.) Serologic work-up was positive for P-ANCA. She required one session of hemodialysis and solumedrol was started. Biopsy showed rapidly progressive glomerulonephritis with necrotizing granulomas and severe interstitial fibrosis and tubulointerstitial atrophy (IFTA). Rituximab 375mg/m^2 4 doses weekly was the induction. She maintained off dialysis and her creatinine stabilized, improving to 3.13 mg/dL over three months. Patient declined maintenance cytotoxic therapy due to concern for lowered immunity during the COVID-19 pandemic. Whenever prednisone was tapered below 10 mg, creatinine would worsen prompting a prolonged course of steroids(12 months).
AAV is a rare condition that can cause rapid renal failure. Treatment includes steroids and immunosuppressive agents, given as induction and maintenance therapies. Glucocorticoids have many side effects, and recent trials evaluate reducing cumulative steroid dose. Our report describes a patient with severe disease that required a longer than usual course of steroids to maintain remission. Her regimen presents some treatment challenges, given the current recommendations to taper steroids off sooner. However, her case is unique, as she declined traditional maintenance immunosuppression , but remained in remission with steroids alone.
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Sekar A, Gandhi P, Sheldon V, Reddy A, Campbell R. A Case of Maintenance Prednisone in ANCA Glomerulonephritis. Advances in Clinical Medical Research and Healthcare Delivery. 2023; 3(1). doi: 10.53785/2769-2779.1140.