1989 - 2019年日本快速进展性肾小球肾炎患者的生存及肾脏预后的时间变化
Temporal changes of the life and renal prognoses of patients with rapidly progressive glomerulonephritis in Japan, 1989-2019.
作者信息
Nakajima Kentaro, Kaneko Shuzo, Usui Joichi, Tsuboi Naotake, Sugiyama Hitoshi, Maruyama Shoichi, Isaka Yoshitaka, Narita Ichiei, Yamagata Kunihiro
机构信息
Department of Nephrology, Institute of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan.
Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Japan.
出版信息
Clin Exp Nephrol. 2025 Mar 25. doi: 10.1007/s10157-025-02643-6.
BACKGROUND
This study is a continuation of the Japan Rapidly Progressive GlomeruloNephritis (RPGN) Working Group's chronological nationwide survey.
METHODS
We analyzed 1,660 RPGN cases from 2016-2019 and compared them to 4,179 cases from five earlier periods (1989-1998, 1999-2001, 2002-2008, 2009-2011, 2012-2015). Data on causative diseases, clinical severity, 24-month life and renal survival, and treatment details were collected and compared.
RESULTS
The most recent cohort showed an older median age at onset (median age 74 years), with improved serum creatinine levels (median 2.5 mg/dL). Cumulative survival at 24 months remained stable (periods 1989-1998, 1999-2001, 2002-2008, 2009-2011, 2012-2015, 2016-2019 were each 72.0%, 72.9%, 77.7%, 83.0%, 84.9%, 83.5%, p < 0.01), while renal survival showed a favorable trend in the most recent periods (there were each 68.7%, 75.4%, 76.7%, 73.4%, 78.2%, 78.4%, p < 0.01). Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV)-RPGN had similar outcomes to the overall cohort. Increased rituximab use was observed, with no significant differences in life and renal prognosis between rituximab (RIX) and cyclophosphamide (CY). In severe renal impairment (Cre ≥ 6), renal prognosis was better in the CY or RIX use group than in the non-use group (p = 0.035, 0.025). Anti-glomerular basement membrane disease had a poorer renal prognosis compared to other causes.
CONCLUSIONS
Despite an increasingly older age of onset, both life and renal prognoses for new-onset AAV-RPGN from 2016 to 2019 remain comparable to the best in previous surveys, due to the impact of constant improvements in early diagnosis and changes in treatment.
背景
本研究是日本急进性肾小球肾炎（RPGN）工作组全国性时间序列调查的延续。
方法
我们分析了2016 - 2019年的1660例RPGN病例，并将其与之前五个时期（1989 - 1998年、1999 - 2001年、2002 - 2008年、2009 - 2011年、2012 - 2015年）的4179例病例进行比较。收集并比较了关于致病疾病、临床严重程度、24个月生存率和肾脏生存率以及治疗细节的数据。
结果
最近一组患者发病时的中位年龄较大（中位年龄74岁），血清肌酐水平有所改善（中位值2.5mg/dL）。24个月时的累积生存率保持稳定（1989 - 1998年、1999 - 2001年、2002 - 2008年、2009 - 2011年、2012 - 2015年、2016 - 2019年分别为72.0%、72.9%、77.7%、83.0%、84.9%、83.5%，p < 0.01），而肾脏生存率在最近几个时期呈现出良好的趋势（分别为68.7%、75.4%、76.7%、73.4%、78.2%、78.4%，p < 0.01）。抗中性粒细胞胞浆抗体相关性血管炎（AAV）-RPGN的结果与总体队列相似。观察到利妥昔单抗的使用增加，利妥昔单抗（RIX）和环磷酰胺（CY）在生存率和肾脏预后方面无显著差异。在严重肾功能损害（Cre≥6）时，CY或RIX使用组的肾脏预后优于未使用组（p = 0.035，0.025）。与其他病因相比，抗肾小球基底膜病的肾脏预后较差。
结论
尽管发病年龄越来越大，但由于早期诊断的不断改善和治疗方法的改变，2016年至2019年新发AAV - RPGN的生存率和肾脏预后与之前调查中的最佳结果相当。
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