Basta-Jovanović Gordana, Radojević-Skodrić Sanja, Jovanović Milena, Bogdanović Ljiljana, Bogdanović Radovan, Lezaić Visnja, Nesić Vidosava, Dikman Steven
Srp Arh Celok Lek. 2008 Dec;136 Suppl 4:282-6. doi: 10.2298/sarh08s4282b.
Two types of hereditary nephritis, nonprogressive and progressive, clinically present as asymptomatic haematuria, sometimes combined with proteinuria. At the onset, in both types, light microscopic changes are minimal, immunofluorescence findings are negative, and diagnosis can be made only upon electron microscopic findings that are considered to be specific.
The aim of this study was to determine the significance of Goodpasture antigen detection in diagnosis of progressive and nonprogressive hereditary nephritis in its early phase.
Analysis of renal biopsy specimens was done in patients with hereditary nephritis that were followed from 1990 to 2005. Progression of renal disease was examined in 14 patients with Alport's syndrome, 10 patients with thin basement membrane disease, and 6 patients with unclassified hereditary nephritis diagnosed. For all these cases, indirect immunofluorescence study with serum from a patient with high titer of Goodpasture autoantibodies that recognize the antigenic determinants in human glomerular and tubular basement membrane was performed.
In 11 out of 14 cases diagnosed as Alport's syndrome, there was negative staining with Goodpasture serum, and in 3 additional cases with Alport's syndrome, expression of Goodpasture antigen in glomerular basement membrane and thin basement membrane was highly reduced. In all 10 patients with thin basement membrane disease, immunofluorescence showed intensive, bright linear staining with Goodpasture serum along glomerular and tubular basement membrane. In 2 out of 6 patients with unclassified hereditary nephritis, Goodpasture antigen expression was very strong, in one patient it was very reduced, and in 3 patients it was negative.
The results of our study show that Goodpasture antigen detection plays a very important role in differential diagnosis of progressive and nonpregressive hereditary nephritis, particularly in early phases of the disease.
遗传性肾炎有非进行性和进行性两种类型，临床上表现为无症状血尿，有时合并蛋白尿。发病初期，两种类型的光镜下改变均很轻微，免疫荧光检查结果为阴性，仅根据被认为具有特异性的电镜检查结果才能做出诊断。
本研究旨在确定检测Goodpasture抗原在进行性和非进行性遗传性肾炎早期诊断中的意义。
对1990年至2005年随访的遗传性肾炎患者的肾活检标本进行分析。对14例阿尔波特综合征患者、10例薄基底膜肾病患者和6例诊断为未分类遗传性肾炎的患者进行了肾脏疾病进展情况检查。对所有这些病例，使用来自一名高滴度Goodpasture自身抗体患者的血清进行间接免疫荧光研究，该抗体可识别人类肾小球和肾小管基底膜中的抗原决定簇。
在14例诊断为阿尔波特综合征的病例中，11例用Goodpasture血清染色为阴性，另外3例阿尔波特综合征病例中，肾小球基底膜和薄基底膜中Goodpasture抗原的表达高度降低。在所有10例薄基底膜肾病患者中，免疫荧光显示Goodpasture血清沿肾小球和肾小管基底膜呈强烈、明亮的线性染色。在6例未分类遗传性肾炎患者中，2例Goodpasture抗原表达非常强，1例表达非常降低，3例为阴性。
我们的研究结果表明，检测Goodpasture抗原在进行性和非进行性遗传性肾炎的鉴别诊断中起着非常重要的作用，尤其是在疾病的早期阶段。
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