Ferluga D, Jerse M, Vizjak A, Hvala A, Rozman B, Kos-Golja M, Bren A F
Institute of Pathology, Medical Faculty, University of Ljubljana, Slovenia.
Wien Klin Wochenschr. 2000 Aug 25;112(15-16):692-701.
In addition to the conventional World Health Organization (WHO) classification of lupus glomerulonephritis (GN), various concomitant approaches have been introduced in the evaluation of renal biopsies of patients with systemic lupus erythematosus (SLE) in order to increase the impact of biopsies on the decision concerning the most appropriate therapy as well as for establishing the prognosis. Three hundred and seventy kidney tissue samples from 267 SLE patients were analysed using standardised light, electron and immunofluorescence microscopic techniques. In 155 patients, a comparative clinical follow-up study and statistical analysis were performed. The study highlighted the heterogeneity of WHO classes IV and III, which include 5 and 6 different conventional histomorphologic types of GN, respectively. Mixed membranous and proliferative GN associated with "full-house" mesangial-transmembranous immune deposits, demonstrated in more than one third of our SLE cases, appears to be diagnostically most characteristic. Immune deposits distributed in the glomeruli in five different patterns, obviously play a major role in the pathogenesis of various WHO classes and histomorphologic types of lupus GN. Additional mechanisms related to the occurrence of antiphospholipid antibodies and antineutrophil cytoplasmic antibodies are suggested to contribute to the histomorphologic heterogeneity of WHO class III and IV lupus GN, particularly to the development of thrombotic, necrotising and crescentic glomerular lesions. In the present study, a statistically significant association was demonstrated between increasing mean values of the activity index and glomerular deposit distribution patterns labeled by subendothelial deposits. Furthermore, a significant correlation was established between an increasing risk of developing renal failure and increasing mean values of the chronicity index. Differences in the increasing risk of developing renal failure between groups with different histomorphologic types of GN and different immune deposit distribution patterns were not statistically significant. The surprisingly high renal survival rate of more than 80% noted in lupus patients with predominantly necrotising crescentic GN during the mean follow-up period of 40 months appears to be related to the more aggressive treatment of those patients. Our study confirmed a significant role of the WHO classification of lupus GN in the decision concerning the most appropriate treatment and prognostication. An increasing risk of irreversible renal failure in patients with WHO class IV lesions in contrast to those of WHO class III and in contrast to those of the category incorporating all other WHO classes was shown to be statistically significant.
除了传统的世界卫生组织（WHO）狼疮性肾炎（GN）分类外，为了增强肾活检对确定最恰当治疗方案及判断预后的影响，在系统性红斑狼疮（SLE）患者肾活检评估中引入了多种辅助方法。使用标准化的光镜、电镜和免疫荧光显微镜技术对267例SLE患者的370份肾组织样本进行了分析。对155例患者进行了对比临床随访研究和统计分析。该研究突出了WHO分类中IV类和III类的异质性，其中IV类和III类分别包含5种和6种不同的传统组织形态学类型的GN。在超过三分之一的SLE病例中出现的与“满堂亮”系膜-跨膜免疫沉积物相关的混合性膜性和增生性GN，在诊断上似乎最具特征性。免疫沉积物以五种不同模式分布于肾小球，显然在各种WHO分类及组织形态学类型的狼疮性GN的发病机制中起主要作用。与抗磷脂抗体和抗中性粒细胞胞浆抗体产生相关的其他机制被认为促成了WHO III类和IV类狼疮性GN的组织形态学异质性，尤其是血栓形成、坏死性和新月体性肾小球病变的发生。在本研究中，活动指数平均值增加与以内皮下沉积物标记的肾小球沉积物分布模式之间显示出统计学上的显著关联。此外，肾衰竭发生风险增加与慢性指数平均值增加之间建立了显著相关性。不同组织形态学类型的GN组和不同免疫沉积物分布模式组在肾衰竭发生风险增加方面的差异无统计学意义。在平均40个月的随访期内，以坏死性新月体性GN为主的狼疮患者中惊人的超过80%的高肾脏存活率似乎与对这些患者更积极的治疗有关。我们的研究证实了WHO狼疮性GN分类在确定最恰当治疗方案和判断预后方面的重要作用。与WHO III类患者以及包含所有其他WHO类别的患者相比，WHO IV类病变患者不可逆肾衰竭的发生风险增加具有统计学显著性。
