Epidemiology and temporal changes in the prognosis of rapidly progressive glomerulonephritis in Japan: a nationwide 1989?2015 survey

Epidemiology and temporal changes in the prognosis of rapidly progressive glomerulonephritis in Japan: a nationwide 1989?2015 survey
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日本急进性肾小球肾炎的流行病学和预后的时间变化:1989年至2015年全国调查

DOI:
10.1007/s10157-021-02148-y
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发表时间:
2021
影响因子:
2.3
通讯作者:
Narita Ichiei
Narita Ichiei
中科院分区:
医学4区
文献类型:
--
作者:
Kaneko Shuzo;Yamagata Kunihiro;Usui Joichi;Tsuboi Naotake;Sugiyama Hitoshi;Maruyama Shoichi;Narita Ichiei

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研究背景快速进展性肾小球肾炎(RPGN)可在短期内进展为终末期肾病。这项研究是日本RPGN工作组进行的全国性按时间顺序调查的延续。MethodsWe检查了四个时期(1989-1998,1999-2001,2002-2008,2009-2011)登记的2793例RPGN病例,以及2012-2015年的1386例病例。作为潜在的预后决定因素,我们调查了发病期,临床严重程度(CS)等级[根据年龄,血清肌酐(sCr)和C-反应蛋白水平,以及是否存在肺部病变分类]和病因疾病。(72.0%,72.9%,77.7%,83.0%,84.9%,p < 0.001)。累积肾存活率在最后一个时期也有所改善(趋势为68.7%、75.4%、76.7%、73.4%、78.2%,p < 0.001)。CS分级被很好地分层以预测生活和肾脏疾病。抗肾小球基底膜疾病(aGBMD)-RPGN的肾脏预后比其他疾病差。在抗中性粒细胞胞浆抗体相关性血管炎(AAV-RPGN,占总体RPGN的> 70%)中,预后结果与总体RPGN相似。sCr < 3 mg/dL者近期肾功能预后较好(24个月累积肾功能存活率为97.9%),sCr ≥ 3 mg/dL者近期肾功能存活率为61.5%。aGBMD-RPGN的肾脏预后没有改善。
BackgroundRapidly progressive glomerulonephritis (RPGN) can progress to end-stage kidney disease within a short period. This study is a continuation of the chronological nationwide survey conducted by the Japan-RPGN working group.MethodsWe examined a total of 2793 RPGN cases registered during four periods (1989–1998, 1999–2001, 2002–2008, 2009–2011) plus 1386 cases in 2012–2015. As potential prognostic determinants, we investigated the onset period, the clinical severity (CS) grade [classified according to age, serum creatinine (sCr) and C-reactive protein levels, and presence/absence of lung lesions], and causative disease.ResultsThe cumulative overall RPGN patient survival at 24 months kept improving over the five periods (72.0%, 72.9%, 77.7%, 83.0%, 84.9%,p< 0.001 for trend). The cumulative renal survival also improved in the latest period (68.7%, 75.4%, 76.7%, 73.4%, 78.2%,p< 0.001 for trend). The CS grade was well stratified to predict both life and renal prognoses. Anti-glomerular basement membrane disease (aGBMD)-RPGN had a poorer renal prognosis than other diseases. In anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV-RPGN, accounting for > 70% of the overall RPGN), the prognostic results were similar to that for overall RPGN. There was a much better renal prognosis for the latest period under the condition of sCr < 3 mg/dL (the 24-month cumulative renal survival: 97.9%), but not for sCr ≥ 3 mg/dL (61.5%).ConclusionsIn overall RPGN as well as AAV-RPGN, both life and renal prognoses tended to improve, but the favorable renal result was substantially limited to mild cases. There was no improvement of the renal prognosis in aGBMD-RPGN.