Salehi Tania, Chapman Gavin B, Farrah Tariq E, Chapman Fiona A, Pugh Dan, Hunter Robert W, Dhaun Neeraj
Department of Renal Medicine, Royal Infirmary of Edinburgh, Edinburgh, UK.
Central and Northern Adelaide Renal and Transplantation Service, Adelaide, South Australia, Australia.
Kidney Int Rep. 2025 Apr 22;10(7):2334-2343. doi: 10.1016/j.ekir.2025.04.030. eCollection 2025 Jul.
The optimal duration of immunosuppressive therapy for antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is uncertain. Glucocorticoids are a mainstay of treatment but are associated with significant morbidity. Here, we describe outcomes of a cohort of patients treated with combination cyclophosphamide and rituximab induction alongside a rapidly tapering oral-only glucocorticoid regimen.
In this retrospective, observational, cohort study, we identified patients presenting with AAV between 2011 and 2023 treated with combination cyclophosphamide and rituximab induction therapy. We analyzed biochemical, histologic, and outcome data, including time-to-remission and relapse rate. A subgroup analysis compared outcomes based on glucocorticoid duration.
A total of 112 patients with active AAV treated with combination cyclophosphamide and rituximab were identified (median age: 67 years; 85% kidney involvement; baseline estimated glomerular filtration rate [eGFR] 24 ml/min per 1.73 m). Of the patients, 96% achieved remission; median time-to-remission was 77 (interquartile range [IQR]: 64-92) days. All patients demonstrated biochemical and histologic improvement following treatment. Five patients (5%) experienced a disease relapse over 2.9 (IQR: 1.7-4.3) years follow-up. The cumulative glucocorticoid dose was 1780 (IQR: 1141-2935) mg with median duration of 12.5 (IQR: 8.0-39.0) weeks. Patients treated with oral glucocorticoids for > 12 weeks received a higher cumulative dose (2935 vs. 1133 mg; < 0.001) with a trend toward more serious infections (21% vs. 7%; = 0.06) than those treated for 12 weeks with no differences in disease remission (100% vs. 91%; = 0.07) or relapse (9% vs. 0%; = 0.07) rates.
Early withdrawal of oral glucocorticoid therapy in patients with severe AAV treated with combination cyclophosphamide and rituximab induction immunosuppression is safe and effective and may reduce morbidity, in particular serious infections.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）免疫抑制治疗的最佳持续时间尚不确定。糖皮质激素是治疗的主要药物，但会带来显著的发病率。在此，我们描述了一组接受环磷酰胺和利妥昔单抗联合诱导治疗以及快速减量的单纯口服糖皮质激素方案的患者的治疗结果。
在这项回顾性观察队列研究中，我们确定了2011年至2023年间接受环磷酰胺和利妥昔单抗联合诱导治疗的AAV患者。我们分析了生化、组织学和治疗结果数据，包括缓解时间和复发率。亚组分析比较了基于糖皮质激素持续时间的治疗结果。
共确定了112例接受环磷酰胺和利妥昔单抗联合治疗的活动性AAV患者（中位年龄：67岁；85%有肾脏受累；基线估计肾小球滤过率[eGFR]为24 ml/min/1.73 m²）。其中，96%的患者实现缓解；中位缓解时间为77（四分位间距[IQR]：64 - 92）天。所有患者治疗后生化和组织学均有改善。5例患者（5%）在2.9（IQR：1.7 - 4.3）年的随访中出现疾病复发。糖皮质激素累积剂量为1780（IQR：1141 - 2935）mg，中位持续时间为12.5（IQR：8.0 - 39.0）周。口服糖皮质激素治疗超过12周的患者累积剂量更高（2935 vs. 1133 mg；P < 0.001），严重感染的趋势更高（21% vs. 7%；P = 0.06），但疾病缓解率（100% vs. 91%；P = 0.07）或复发率（9% vs. 0%；P = 0.07）无差异。
对于接受环磷酰胺和利妥昔单抗联合诱导免疫抑制治疗的重症AAV患者，早期停用口服糖皮质激素治疗是安全有效的，且可能降低发病率，尤其是严重感染的发病率。
