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. 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 m 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.