ANCA-associated systemic vasculitis in Japan: clinical features and prognostic changes
ANCA-associated systemic vasculitis in Japan: clinical features and prognostic changes
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DOI：
10.1007/s10157-012-0598-2
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发表时间：
2012-08-01
影响因子：
2.3
通讯作者：
Matsuo, Seiichi
中科院分区：
文献类型：
--
作者：
Yamagata, Kunihiro;Usui, Joichi;Matsuo, Seiichi
日本抗中性粒细胞胞浆抗体（ANCA）相关血管炎/快速进行性肾小球肾炎（AAV/RPGN）患者预后较差，因此本研究旨在规范治疗并确定患者和肾脏预后。本回顾性队列研究的参与者为824例anca阳性RPGN患者，其中705例仅髓过氧化物酶(MPO)-ANCA阳性。在早期队列（A组；1988年至1998年之间诊断的病例）中，患者经常因机会性感染而死亡。因此，我们建议减少强的松龙剂量（口服强的松龙剂量< 0.8 mg/kg/天），联合或不联合环磷酰胺，用于日本RPGN患者的初始治疗。在此推荐后，患者的1年生存率提高：A组为75%，B组为79%（1999 - 2002年），C组为81%（2003年以后）。在整个观察期内，治疗开始时平均血清肌酐水平下降，1年肾脏生存率也有所改善（A组为72%，B组为83%，C组为83%），而C组的复发率明显升高（A组0.05/患者-年，B组0.07/患者-年，C组0.13/患者-年）。口服强的松龙剂量< 0.8 mg/kg/天，联合或不联合环磷酰胺作为初始治疗，可提高日本老年AAV/RPGN患者的生存率。然而，维持治疗应避免复发，以改善肾脏预后。
This study was conducted to standardize treatment and determine patient and renal outcome in Japanese anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis/rapidly progressive glomerulonephritis (AAV/RPGN) patients, because the prognosis of AAV/RPGN patients in Japan had been poor compared with that of other countries.The participants in this retrospective cohort study were 824 ANCA-positive RPGN patients, 705 of whom were only myeloperoxidase (MPO)-ANCA positive.Among the early-years cohort (group A; cases diagnosed between 1988 and 1998), patients frequently died due to opportunistic infection. Therefore, we recommended a reduced dose of prednisolone (oral prednisolone dose < 0.8 mg/kg/day) with or without cyclophosphamide for initial treatment of Japanese RPGN patients. After this recommendation, 1-year survival of the patients improved: 75% in group A, 79% in group B (between 1999 and 2002), and 81% in group C (after 2003). During the entire observation period, average serum creatinine level at the start of treatment decreased, and improvement of 1-year renal survival was also found (72% in group A, 83% in group B, and 83% in group C), while the recurrence rate was significantly increased in group C (0.05/patient-year in group A, 0.07/patient-year in group B, and 0.13/patient-year in group C).Oral prednisolone dose < 0.8 mg/kg/day with or without cyclophosphamide as an initial treatment could improve patient survival in older Japanese AAV/RPGN patients. However, maintenance treatment avoiding relapse should be established to improve renal outcomes.