Rheumatology Unit, Department of Medicine-DIMED, University Hospital, Padova, Italy.
Biostatistics, Epidemiology and Public Health Unit, Department of Cardiac, Thoracic and Vascular Sciences, University Hospital, Padova, Italy.
Clin Exp Rheumatol. 2020 Mar-Apr;38 Suppl 124(2):201-206. Epub 2020 May 22.
In ANCA-associated vasculitis (AAV), renal relapses are cause of concern as they are unpredictable and predictors of end-stage renal disease (ESRD). We aimed to assess the frequency of major renal (MR) relapses in AAV and to identify independent base-line predictors.
We performed a retrospective monocentric observational cohort study of patients affected by granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and renal limited vasculitis (RLV), diagnosed from 2000 to 2019, and who achieved clinical remission defined as Birmingham Vasculitis Activity Index version 3 (BVASv3)=0 and/or clinical judgment. MR relapse was defined as the occurrence of major items of renal BVASv3. Univariate and multivariable analysis was performed with competitive risk analysis.
We included 96 patients: 73 GPA, 21 MPA and 2 RLV. Eighty-five (90%) patients were ANCA-positive: 56 c-ANCA/PR3, 28 p-ANCA/MPO and 1 double positive. During the follow-up, 17/96 patients developed at least one MR relapse, 2/96 progressed to ESRD and 3/96 died without events; 74 did not develop MR relapse. Patients with MR relapse were all ANCA positive and had higher frequency of skin (p=0.034), kidney (p=0.004) and nervous system (p=0.024) involvement and lower fre¬quency of ear, nose and throat (ENT) manifestations (p=0.043). At multivariable analysis, renal involvement at baseline (sHR 20.4, 95% confidence interval (95% CI) 2.6-158.2, p=0.004) and remission-induction treatment without cyclophosphamide and/or rituximab (sHR 4.2, 95% CI 1.5-12.0, p=0.007) were independent predictors of MR relapses.
Baseline renal involvement predicts MR relapse in AAV while intense initial treatment seems to be protective.
在抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）中，肾脏复发是一个令人担忧的问题，因为它们是不可预测的，并且是终末期肾病（ESRD）的预测因素。我们旨在评估 AAV 中主要肾脏（MR）复发的频率，并确定独立的基线预测因素。
我们进行了一项回顾性单中心观察性队列研究，纳入了 2000 年至 2019 年间诊断为肉芽肿性多血管炎（GPA）、显微镜下多血管炎（MPA）和肾局限性血管炎（RLV）的患者，这些患者达到了临床缓解的定义，即伯明翰血管炎活动指数 3 版（BVASv3）=0 和/或临床判断。MR 复发定义为发生主要肾脏 BVASv3 项目。采用竞争风险分析进行单变量和多变量分析。
我们纳入了 96 名患者：73 名 GPA、21 名 MPA 和 2 名 RLV。85 名（90%）患者为 ANCA 阳性：56 名 c-ANCA/PR3、28 名 p-ANCA/MPO 和 1 名双阳性。在随访期间，17/96 名患者至少发生了一次 MR 复发，2/96 名患者进展为 ESRD，3/96 名患者死亡但无事件发生；74 名患者未发生 MR 复发。发生 MR 复发的患者均为 ANCA 阳性，且皮肤（p=0.034）、肾脏（p=0.004）和神经系统（p=0.024）受累的频率更高，而耳鼻喉（ENT）表现（p=0.043）的频率更低。多变量分析显示，基线时的肾脏受累（sHR 20.4，95%置信区间[95%CI]2.6-158.2，p=0.004）和不使用环磷酰胺和/或利妥昔单抗的缓解诱导治疗（sHR 4.2，95%CI 1.5-12.0，p=0.007）是 MR 复发的独立预测因素。
基线时的肾脏受累可预测 AAV 中的 MR 复发，而强化初始治疗似乎具有保护作用。
