The First Department of Internal Medicine, University of Occupational and Environmental Health Japan, Kitakyushu, Japan.
Graduate School of Health Innovation, Kanagawa University of Human Services, Kawasaki, Japan.
Ann Rheum Dis. 2024 Jan 2;83(1):103-111. doi: 10.1136/ard-2023-224472.
Life-threatening antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with rapidly progressive glomerulonephritis (RPGN) and/or alveolar haemorrhage (AH) has a poor prognosis. Rituximab (RTX) is as effective as cyclophosphamide (CY) in remission induction therapy; however, the effectiveness and safety of RTX have not been established in life-threatening AAV. This study aimed to investigate the short-term effectiveness and safety of RTX in life-threatening AAV with RPGN and/or AH.
Between April 2018 and March 2020, cases treated with systemic glucocorticoids and RTX or intravenous CY (IVCY) was extracted from a Japanese nationwide inpatient database. Effectiveness was evaluated by in-hospital mortality and severe renal dysfunction requiring haemodialysis (HD) at discharge. Safety was evaluated by the in-hospital incidence of infections. The propensity score (PS) for RTX was estimated. Multivariable Cox and logistic regression with adjustment for PS were conducted to estimate the association of RTX with outcomes.
From 16 001 612 hospitalised records, 687 life-threatening AAV cases were extracted. No significant difference in in-hospital mortality (adjusted HR 1.06; 95% CI 0.62 to 1.80) was found between the groups. Although the RTX group had a lower risk of fungal infections (adjusted OR (aOR) 0.45; 95% CI 0.23 to 0.84) and pneumocystis pneumonia (aOR 0.58; 95% CI 0.32 to 1.00), they might have an increased risk of severe renal dysfunction requiring HD at discharge (aOR 2.58; 95% CI 1.02 to 6.91).
In life-threatening AAV, RTX has similar short-term effectiveness on mortality to IVCY. Although RTX might have a lower risk of fungal infections and pneumocystis pneumonia, the short-term renal prognosis might be inferior to IVCY.
伴有快速进展性肾小球肾炎（RPGN）和/或肺泡出血（AH）的危及生命的抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）预后不良。利妥昔单抗（RTX）在缓解诱导治疗中与环磷酰胺（CY）同样有效；然而，RTX 在危及生命的 AAV 中的有效性和安全性尚未得到证实。本研究旨在探讨 RTX 治疗伴有 RPGN 和/或 AH 的危及生命的 AAV 的短期疗效和安全性。
2018 年 4 月至 2020 年 3 月，从日本全国住院患者数据库中提取接受全身糖皮质激素和 RTX 或静脉注射 CY（IVCY）治疗的病例。通过住院死亡率和出院时需要血液透析（HD）的严重肾功能障碍来评估疗效。通过住院期间感染的发生率评估安全性。估计 RTX 的倾向评分（PS）。采用多变量 Cox 回归和调整 PS 的逻辑回归来估计 RTX 与结局的相关性。
从 16001612 份住院记录中提取了 687 例危及生命的 AAV 病例。两组间住院死亡率无显著差异（调整后的 HR 1.06；95%CI 0.62 至 1.80）。尽管 RTX 组真菌感染（调整后的 OR（aOR）0.45；95%CI 0.23 至 0.84）和肺囊虫肺炎（aOR 0.58；95%CI 0.32 至 1.00）的风险较低，但他们可能有更高的出院时需要 HD 的严重肾功能障碍风险（aOR 2.58；95%CI 1.02 至 6.91）。
在危及生命的 AAV 中，RTX 在死亡率方面与 IVCY 的短期疗效相当。尽管 RTX 可能具有较低的真菌感染和肺囊虫肺炎风险，但短期肾功能预后可能不如 IVCY。
