Long-Term Follow-Up of a Glucocorticoid Minimizing Regimen for the Treatment of Severe Antineutrophil Cytoplasmic Autoantibody–Associated Vasculitis

Introduction: Glucocorticoids (GCs) are pivotal in treating antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV); however, their use is associated with significant toxicities. Findings of recent trials support reduced GC dosing, demonstrating efficacy with fewer adverse events....

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Main Authors: Stephen P. McAdoo, Ruth J. Pepper, Maria Prendecki, Amrita Dhutia, Tony Lopez, Aine Burns, Marie Condon, Sally Hamour, Megan Griffith, Jeremy Levy, Tom Cairns, Mark Little, Alan D. Salama, Charles D. Pusey
Format: Article
Language:English
Published: Elsevier 2025-06-01
Series:Kidney International Reports
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Online Access:http://www.sciencedirect.com/science/article/pii/S2468024925001378
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Summary:Introduction: Glucocorticoids (GCs) are pivotal in treating antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV); however, their use is associated with significant toxicities. Findings of recent trials support reduced GC dosing, demonstrating efficacy with fewer adverse events. Methods: This was a retrospective cohort study evaluating long-term outcomes of a rapid taper GC regimen in severe AAV. Fifty-eight patients treated with combination rituximab, low-dose i.v. cyclophosphamide, and a limited course of GC (total equivalent prednisolone: 1148 [887–1390] mg; median duration of GC: 14 [7–15] days) were followed-up with for a median of 41 (interquartile range: 27–48) months. Results: Median Birmingham Vasculitis Activity Score (BVAS), serum creatinine, estimated glomerular filtration rates (eGFR), and urinary protein-to-creatinine ratio (uPCR) at presentation were 14 (12–18), 176 (132–270) μmol/l, 29 (19–50) ml/min per 1.73 m2, and 152 (77–289) mg/mmol, respectively. At 3 months, 51 of 58 (88%) achieved remission without additional GC treatment, and this was sustained in 49 of 56 (88%) at 1 year, with improved kidney parameters (creatinine: 109 [83–162] μmol/l, eGFR: 54 [37–74]ml/min per 1.73 m2, uPCR: 35 [12–94] mg/mmol at 12 months). At 3 years, kidney and overall survival were 33 of 35 (94%) and 35 of 38 (93%), respectively; and 27 of 35 (77%) were in sustained remission without additional GC. Early improvements in disease activity and kidney function—and in long-term kidney and patient survival—were comparable with a previous cohort treated with an equivalent rituximab-cyclophosphamide regimen and a conventional GC taper, whereas adverse events (infection, new-onset diabetes) were less frequent. Conclusion: Ultrarapid GC withdrawal is safe and effective for many patients with AAV when used with combination induction regimens. This approach warrants confirmatory controlled studies and may have a place in the current management of patients at high risk of GC toxicities.
ISSN:2468-0249