利用重复活检和壁层上皮细胞标志物观察微小病变病和局灶节段性肾小球硬化的形态学特征
Morphological Features of Minimal Change Disease and Focal Segmental Glomerulosclerosis Using Repeat Biopsy and Parietal Epithelial Cell Marker.
作者信息
Suzuki Tomo, Kohatsu Kaori, Han Wei, Watanabe Shiika, Yahagi Koichi, Nakata Mayumi, Ueno Toshiharu, Ichikawa Daisuke, Imai Naohiko, Shirai Sayuri, Koike Junki, Shibagaki Yugo
机构信息
Division of Nephrology and Hypertension, Department of Internal Medicine, St. Marianna University School of Medicine, Kanagawa, Japan.
Department of Nephrology, Kameda Medical Center, Chiba, Japan.
出版信息
Kidney Dis (Basel). 2020 Mar;6(2):119-124. doi: 10.1159/000505125. Epub 2020 Jan 31.
INTRODUCTION
Minimal change disease (MCD) and primary focal segmental glomerulosclerosis (FSGS) are representative podocyte diseases. The clinical cause of MCD and FSGS has not been clearly elucidated yet. However, it is important to distinguish MCD and FSGS because their prognoses and responses to treatment are quite different.
OBJECTIVE
This study aimed to examine whether parietal epithelial cell (PEC) marker and repeat biopsy are useful for diagnosing primary FSGS.
METHODS
Clinicopathological features of 17 patients with the nephrotic syndrome, who underwent kidney biopsy ≥2 times from 1975 to 2017, and had MCD or FSGS were analyzed using PAX8. We defined patients with PAX8+ cells as PAX8+ and the remainder as PAX8- patients. Three cases of sample insufficiency and 1 non-steroid-resistant or frequently relapsing case indicated for repeat biopsy were excluded.
RESULTS
Among the 13 patients studied, 4 were PAX8+ and 9 were PAX8- (median age: 41 and 46 years, -respectively, at first biopsy). PAX8+ and PAX8- patients showed no significant differences in clinical data and histological diagnosis except for a significant difference in histological diagnosis at the second biopsy. The number of PAX8+ patients increased to 6. Unlike the first biopsy results, FSGS was present in 5 of 6 (83.3%) PAX8+ patients; MCD occurred in all 7 (100%) PAX8- patients. Three of 6 (50.0%) PAX8+ patients undergoing repeat biopsy were steroid resistant; no (0%) PAX8- patient was steroid resistant. All cases of final FSGS diagnosis were PAX8+ at the first or second biopsy. Only 1 PAX8+ MCD patient was steroid resistant. All PAX8- MCD patients were frequently relapsing.
CONCLUSIONS
More PAX8+ patients were diagnosed with FSGS than PAX8- patients. Clinical presentation of MCD in PAX8- patients was frequently relapsing. PEC marker staining in patients with the nephrotic syndrome, e.g., MCD, may help to diagnose FSGS.
引言
微小病变病（MCD）和原发性局灶节段性肾小球硬化症（FSGS）是典型的足细胞疾病。MCD和FSGS的临床病因尚未完全阐明。然而，区分MCD和FSGS很重要，因为它们的预后和对治疗的反应有很大差异。
目的
本研究旨在探讨壁层上皮细胞（PEC）标志物和重复活检对原发性FSGS诊断是否有用。
方法
对1975年至2017年间接受≥2次肾活检且患有MCD或FSGS的17例肾病综合征患者的临床病理特征进行PAX8分析。我们将有PAX8+细胞的患者定义为PAX8+，其余患者定义为PAX8-。排除3例样本不足和1例因非激素抵抗或频繁复发而需重复活检的病例。
结果
在研究的13例患者中，4例为PAX8+，9例为PAX8-（首次活检时的中位年龄分别为41岁和46岁）。PAX8+和PAX8-患者在临床数据和组织学诊断方面无显著差异，但第二次活检时的组织学诊断有显著差异。PAX8+患者数量增至6例。与首次活检结果不同，6例（83.3%）PAX8+患者中有5例出现FSGS；7例（100%）PAX8-患者均发生MCD。6例接受重复活检的PAX8+患者中有3例（50.0%）对激素抵抗；PAX8-患者中无一例（0%）对激素抵抗。最终诊断为FSGS的所有病例在首次或第二次活检时均为PAX8+。只有1例PAX8+的MCD患者对激素抵抗。所有PAX8-的MCD患者均频繁复发。
结论
诊断为FSGS的PAX8+患者多于PAX8-患者。PAX8-患者的MCD临床表现为频繁复发。肾病综合征患者（如MCD）的PEC标志物染色可能有助于FSGS的诊断。
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