Krämer Stefan, Vogt Kristian, Schreibing Theresa Maria, Busch Martin, Schmitt Tobias, Bergner Raoul, Mosberger Sebastian, Neumann Thomas, Rauen Thomas
Department of Nephrology and Clinical Immunology, RWTH Aachen University Hospital, Pauwelsstraße 30, 52074, Aachen, Germany.
Department of Internal Medicine III, University Hospital Jena, Friedrich-Schiller University, Jena, Germany.
Rheumatol Int. 2024 Dec 24;45(1):7. doi: 10.1007/s00296-024-05757-4.
To explore disease characteristics, renal involvement and induction treatment strategies over the last decades and evaluate relapse rates and renal outcomes in ANCA-associated vasculitides (AAV). We retrospectively analyzed remission, relapse rates and the occurrence of the composite endpoint (comprising death and renal failure) in newly diagnosed AAV cases in four tertial referral centers in Germany and Switzerland diagnosed between 1999 and 2022. Hazard ratios were computed by Cox proportional hazard and Kaplan-Meier curves were plotted to compare therapeutic strategies after propensity-matching. In our cohort of 358 AAV patients, 203 (58.1%) were classified as granulomatosis with polyangiitis (GPA) based on the novel 2022 ACR/EULAR criteria, 139 (38.8%) as microscopic polyangiitis (MPA). The proportion of MPA cases among all AAV patients increased from 28.9% between 1999 and 2013 up to 46.7% thereafter. Cyclophosphamide (CYC) was chosen most frequently for remission induction (74.8% before 2013 and 57.3% thereafter), whereas the use of rituximab (RTX) increased from 5 to 26% within these periods. GPA patients had a higher relapse rate as compared to MPA patients (41.3% vs. 25.9%, p = 0.006). However, in AAV patients with renal involvement, renal events (i.e. end-stage kidney disease or a persistent drop in the estimated glomerular filtration rate (eGFR) below 15 ml/min/1.73 m) occurred more frequently in MPA patients, particularly under RTX treatment as compared to matched CYC counterparts (11.8% vs. 7.5%, p = 0.011). In our cohort, GPA patients exhibited frequent relapses, advocating for a more intense and extended treatment. MPA patients had lower relapse rates, however, RTX was less effective to prevent renal endpoints in MPA as compared to CYC, highlighting the need to further investigate additional treatment strategies.
为探究过去几十年间抗中性粒细胞胞浆抗体相关性血管炎（AAV）的疾病特征、肾脏受累情况及诱导治疗策略，并评估其复发率和肾脏结局。我们回顾性分析了1999年至2022年间在德国和瑞士四个三级转诊中心新诊断的AAV病例的缓解情况、复发率及复合终点（包括死亡和肾衰竭）的发生情况。通过Cox比例风险模型计算风险比，并绘制Kaplan-Meier曲线以比较倾向匹配后的治疗策略。在我们的358例AAV患者队列中，根据2022年美国风湿病学会/欧洲抗风湿病联盟（ACR/EULAR）新标准，203例（58.1%）被分类为肉芽肿性多血管炎（GPA），139例（38.8%）为显微镜下多血管炎（MPA）。MPA病例在所有AAV患者中的比例从1999年至2013年间的28.9%增加至此后的46.7%。环磷酰胺（CYC）最常被用于诱导缓解（2013年前为74.8%，此后为57.3%），而利妥昔单抗（RTX）的使用在这些时期内从5%增加至26%。与MPA患者相比，GPA患者的复发率更高（41.3%对25.9%，p = 0.006）。然而，在有肾脏受累的AAV患者中，MPA患者发生肾脏事件（即终末期肾病或估计肾小球滤过率（eGFR）持续降至15 ml/min/1.73 m²以下）的频率更高，尤其是与匹配的CYC治疗组相比，接受RTX治疗时（11.8%对7.5%，p = 0.011）。在我们的队列中，GPA患者复发频繁，主张采用更强化和延长的治疗。MPA患者复发率较低，然而，与CYC相比，RTX在预防MPA患者的肾脏终点方面效果较差，这突出表明需要进一步研究其他治疗策略。
