Weidner Sven, Geuss Steffen, Hafezi-Rachti Syrus, Wonka Andrea, Rupprecht Harald D
Policlinic of Internal Medicine, Ludwig-Maximilians-University, Munich, Germany.
Nephrol Dial Transplant. 2004 Jun;19(6):1403-11. doi: 10.1093/ndt/gfh161. Epub 2004 Apr 6.
The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are a group of heterogeneous diseases. This study was undertaken to investigate the outcome of Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and renal-limited vasculitis (RLV). Furthermore, we analysed the differences in patients with proteinase 3-ANCA (PR3-ANCA) and those with myeloperoxidase-ANCA (MPO-ANCA), which have not been assessed in a homogeneously treated group of patients with renal involvement.
In this retrospective analysis, 80 patients with a new diagnosis of WG, MPA or RLV with biopsy-proven renal involvement were followed over a median of 46.7 months (range: 0.8-181.9 months). All patients had induction treatment with cyclophosphamide and oral corticosteroids.
At the end of follow-up, 23% were dependent on dialysis. Renal survival was significantly worse in patients with WG compared with patients with MPA or RLV (P = 0.04). A higher rate of end-stage renal disease (ESRD) was noticed in PR3-ANCA- vs MPO-ANCA-positive patients. A total of 21 patients (26%) died. Predictors of patient mortality were development of ESRD, older age and the maximum creatinine in the first month. Mortality was found to be higher in patients with WG and was significantly higher in PR3-ANCA-positive cases (P = 0.02). The relative risk of death was 9.32 times higher in PR3-ANCA- vs MPO-ANCA-positive patients.
Our data underscore the pathogenetic potential of ANCA by demonstrating a more aggressive disease state and a poorer outcome in patients with PR3-ANCA.
抗中性粒细胞胞浆抗体（ANCA）相关血管炎是一组异质性疾病。本研究旨在调查韦格纳肉芽肿（WG）、显微镜下多血管炎（MPA）和肾脏局限性血管炎（RLV）的预后。此外，我们分析了蛋白酶3-ANCA（PR3-ANCA）阳性患者和髓过氧化物酶-ANCA（MPO-ANCA）阳性患者之间的差异，此前尚未在一组接受统一治疗的肾脏受累患者中对此进行评估。
在这项回顾性分析中，对80例新诊断为WG、MPA或RLV且经活检证实有肾脏受累的患者进行了中位时间为46.7个月（范围：0.8 - 181.9个月）的随访。所有患者均接受了环磷酰胺和口服糖皮质激素的诱导治疗。
随访结束时，23%的患者依赖透析。与MPA或RLV患者相比，WG患者的肾脏存活率显著更差（P = 0.04）。PR3-ANCA阳性患者的终末期肾病（ESRD）发生率高于MPO-ANCA阳性患者。共有21例患者（26%）死亡。患者死亡的预测因素为ESRD的发生、年龄较大以及第一个月的最高肌酐水平。发现WG患者的死亡率较高，并在PR3-ANCA阳性病例中显著更高（P = 0.02）。PR3-ANCA阳性患者与MPO-ANCA阳性患者相比，死亡相对风险高9.32倍。
我们的数据通过证明PR3-ANCA阳性患者疾病状态更具侵袭性且预后更差，强调了ANCA的致病潜力。
