Histologic subclassification of IgA nephropathy: A clinicopathologic study of 244 cases

Histologic subclassification of IgA nephropathy: A clinicopathologic study of 244 cases
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DOI:
10.1016/s0272-6386(97)90456-x
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发表时间:
1997-06-01
影响因子:
13.2
通讯作者:
Haas, M
Haas, M
中科院分区:
医学1区
文献类型:
--
作者:
Haas, M

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伊加肾病(IgAN)在肾活检时可表现出多种组织学模式,从最小病变到弥漫性增生性肾小球肾炎(GN)。244例IgA肾病的组织学特点回顾1980年至1994年诊断的10例肾性肾炎(不包括Schonlein-Henoch肾炎),并使用以下相对简单的组织学分类模式对每个病例进行亚分类:(39例):极轻微或无系膜细胞增生,无肾小球硬化; II类(18例):局灶性节段性肾小球硬化,无活跃的细胞增殖; III类(110例):局灶性增生性GN; IV亚类(42例):弥漫增生性GN;(35例):任何活检显示大于或等于40%的整体肾小球硬化和/或大于或等于40%的估计皮质小管萎缩或丢失。随后对109例在1992年之前或期间接受活检的患者的肾存活率进行分析,这些患者的数据可用,显示组织学亚类与肾存活率之间存在强的统计学显著相关性,顺序为I,II(最大生存期)> III > IV,V,新月体是III类肾存活的一个显著的阴性预后指标(但不在IV亚类中),间质扩张是III和IV亚类中的阴性预后指标,尽管在活检时控制血清肌酐后,这些指标的统计学显著性没有维持。外周肾小球毛细血管沉积物的存在在超微结构上没有预后意义。高血压(收缩压大于或等于130 mm Hg,舒张压大于或等于90 mm Hg)和蛋白尿大于或等于2.0 g/24 hr是肾存活率的显著阴性预后指标,即使在肾活检时控制了血清肌酐。肉眼血尿的存在与单因素分析肾存活率的增加显著相关,但在肾活检时控制血清肌酐时则不相关。本研究的结果证实了IgAN的各种临床和组织病理学表现,并表明了所提出的组织学分类方案在评估患者最终发展为终末期肾病的可能性中的实用性。(C)1997年由国家肾脏基金会,公司。
IgA nephropathy (IgAN) may present with a wide variety of histologic patterns on renal biopsy, ranging from a minimal lesion to a diffuse proliferative glomerutonephritis (GN). The histologic features of 244 cases of IgAN (not including Schonlein-Henoch nephritis) diagnosed between 1980 and 1994 were reviewed, and each case was subclassified using the following, relatively simple histologic classification schema: subclass I (39 cases): minimal or no mesangial hypercellularity, without glomerular sclerosis; subclass II (18 cases): focal and segmental glomerular sclerosis without active cellular proliferation; subclass III (110 cases): focal proliferative GN; subclass IV (42 cases): diffuse proliferative GN; and subclass V (35 cases): any biopsy showing greater than or equal to 40% globally sclerotic glomeruli and/or greater than or equal to 40% estimated cortical tubular atrophy or loss. Subsequent analysis of renal survival in 109 patients who underwent biopsy before or during 1992 for whom such data were available showed a strong, statistically significant correlation between histologic subclass and renal survival, with an order I, II (greatest survival) > III > IV, V, Crescents were a significant negative prognostic indicator for renal survival in subclass III (but not in subclass IV), and interstitial expansion was a negative prognostic indicator in subclasses III and IV, although the statistical significance of these were not maintained after controlling for serum creatinine at the time of biopsy, The presence of peripheral glomerular capillary deposits ultrastructurally had no prognostic significance, With respect to clinical presentation, hypertension (systolic blood pressure greater than or equal to 130 mm Hg and diastolic blood pressure greater than or equal to 90 mm Hg) and proteinuria of greater than or equal to 2.0 g/24 hr were significant negative prognostic indicators for renal survival, even when controlling for serum creatinine at the time of renal biopsy. The presence of gross hematuria correlated significantly with increased renal survival by univariate analysis, but not when controlling for serum creatinine at the time of renal biopsy, The findings of this study confirm the wide variety of clinical and histopathologic presentations of IgAN, and indicate the utility of the proposed histologic classification schema in assessing a patient's likelihood of ultimately developing end-stage renal disease. (C) 1997 by the National Kidney Foundation, Inc.