抗中性粒细胞胞浆抗体相关肾小球肾炎的肾脏预后指标。

An index for renal outcome in ANCA-associated glomerulonephritis.

作者信息

Vergunst Clarissa E, van Gurp Eveline, Hagen E Christiaan, van Houwelingen Hans C, Hauer Herbert A, Noël Laure-Hélène, Waldherr Rüdiger, Ferrario Franco, van der Woude Fokko J, Bruijn Jan A, Bajema Ingeborg M

机构信息

Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands.

出版信息

Am J Kidney Dis. 2003 Mar;41(3):532-8. doi: 10.1053/ajkd.2003.50115.

DOI:10.1053/ajkd.2003.50115

PMID:12612975

Abstract

BACKGROUND

The aim of this study is to analyze the predictive value of clinical, serological, and histological parameters for renal outcome in antineutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis by multivariate analysis and create an index valid for clinical use.

METHODS

Data from 160 patients with Wegener's granulomatosis, microscopic polyangiitis, and idiopathic rapidly progressive glomerulonephritis without immune deposits (renal-limited vasculitis) were collected. The Cockcroft formula was used to assess renal function expressed by glomerular filtration rate (GFR) at the time of renal biopsy (t = 0) and 1 year later (t = 1). Other clinical parameters were age, sex, and diagnosis. ANCA test results were scored as cytoplasmic ANCA/antiproteinase 3 (anti-PR3) or perinuclear ANCA/antimyeloperoxidase (anti-MPO) positive or negative. Histological data included normal glomeruli, fibrinoid necrosis, extracapillary proliferation, granulomas, interstitial edema, focal and diffuse infiltrates, fibrosis, tubular cylinders/casts, tubular atrophy, tubular necrosis, sclerosis, mesangial proliferation, mesangial matrix expansion, arteriosclerosis, and infiltrates in arterioles. In a separate analysis, we explored whether there were histological differences between patients with anti-PR3 and anti-MPO ANCA test results.

RESULTS

Forty percent of the variation in renal function at the time of biopsy can be explained by the presence or absence of tubular atrophy, normal glomeruli, fibrinoid necrosis, extracapillary proliferation, and age. Renal function at the time of biopsy is the best predictor for renal function at t = 1 in patients with ANCA-associated glomerulonephritis. Together with normal glomeruli, fibrinoid necrosis, and age, it explains more than 60% of the variation in GFR at t = 1. ANCA subtype has no independent contribution in predicting patient prognosis. Results translated into a clinically relevant index: GFR at t = 1 = 36.96 + 0.65* (GFR at t = 0) + 10.52 (if normal glomeruli present) + 7.72 (if fibrinoid necrosis present) - 0.42* (age).

CONCLUSION

The index created with results from this study provides an indication of renal outcome in patients diagnosed with ANCA-associated glomerulonephritis.

摘要

背景

本研究旨在通过多变量分析，分析临床、血清学和组织学参数对抗中性粒细胞胞浆抗体（ANCA）相关性肾小球肾炎肾脏预后的预测价值，并创建一个可用于临床的指标。

方法

收集了160例韦格纳肉芽肿、显微镜下多血管炎和无免疫沉积物的特发性快速进展性肾小球肾炎（肾局限性血管炎）患者的数据。采用Cockcroft公式评估肾活检时（t = 0）和1年后（t = 1）以肾小球滤过率（GFR）表示的肾功能。其他临床参数包括年龄、性别和诊断。ANCA检测结果分为胞浆型ANCA/抗蛋白酶3（抗PR3）或核周型ANCA/抗髓过氧化物酶（抗MPO）阳性或阴性。组织学数据包括正常肾小球、纤维蛋白样坏死、毛细血管外增生、肉芽肿、间质水肿、局灶性和弥漫性浸润、纤维化、肾小管管型/铸型、肾小管萎缩、肾小管坏死、硬化、系膜增生、系膜基质扩张、动脉硬化以及小动脉浸润。在另一项分析中，我们探讨了抗PR3和抗MPO ANCA检测结果不同的患者之间是否存在组织学差异。

结果

活检时肾功能变化的40%可由肾小管萎缩、正常肾小球、纤维蛋白样坏死、毛细血管外增生的有无以及年龄来解释。活检时的肾功能是ANCA相关性肾小球肾炎患者t = 1时肾功能的最佳预测指标。与正常肾小球、纤维蛋白样坏死和年龄一起，它解释了t = 1时GFR变化的60%以上。ANCA亚型在预测患者预后方面没有独立作用。结果转化为一个具有临床相关性的指标：t = 1时的GFR = 36.96 + 0.65×（t = 0时的GFR）+ 10.52（如果存在正常肾小球）+ 7.72（如果存在纤维蛋白样坏死）- 0.42×（年龄）。