Clinical and pathologic characteristics of pauci-immune anti-myeloperoxidase antibody associated glomerulonephritis with nephrotic range proteinuria.
Clinical and pathologic characteristics of pauci-immune anti-myeloperoxidase antibody associated glomerulonephritis with nephrotic range proteinuria.
复制标题
寡免疫抗髓过氧化物酶抗体相关性肾小球肾炎伴肾病范围蛋白尿的临床和病理特征。
DOI：
10.1080/0886022x.2018.1487865
复制
发表时间：
2018-11
期刊：
影响因子：
3
通讯作者：
Yan TK
中科院分区：
文献类型：
--
作者：
Xu PC;Chen T;Gao S;Hu SY;Wei L;Yan TK
工作背景：抗神经细胞胞浆抗体（ANCA）相关性肾小球肾炎（GN）的大量蛋白尿通常被认为与肾活检中的免疫沉积有关。无免疫沉积的肾病ANCA GN（少免疫）是罕见的，尚未进行专门研究。本研究将调查这些患者的特征。研究方法：分析20例经肾活检证实的抗髓过氧化物酶抗体相关性肾小球肾炎伴肾病蛋白尿患者的临床和病理特征，并与无肾病蛋白尿的ANCA GN患者进行比较。结果如下：急性肾损伤（阿基）和肉眼血尿更为常见，但肾外受累在伴肾病蛋白尿的低免疫ANCA GN中的发生率低于不伴肾病蛋白尿的低免疫ANCA GN。两组患者均未出现更严重的低白蛋白血症、高凝状态、高脂血症及血栓形成。与无肾病蛋白尿的患者相比，肾病蛋白尿患者的组织病理学中新月体类型更常见。治疗后蛋白尿迅速下降，但在伴有肾病蛋白尿的免疫缺陷型ANCA GN中发现肾脏预后更差。尿白蛋白/总蛋白比值和尿蛋白电泳结果显示，免疫缺陷型ANCA GN伴肾病蛋白尿患者有明显的非选择性蛋白尿。结论：与无肾病蛋白尿的患者相比，伴肾病蛋白尿的少免疫ANCA GN患者并没有更严重的低白蛋白血症、高凝状态或高脂血症。非选择性蛋白尿可能是原因。然而，尽管对蛋白尿治疗的敏感性良好，但伴肾病蛋白尿的低免疫ANCA GN在组织病理学上具有更常见的新月体类型，阿基和肉眼血尿的发生率更高，肾脏预后更差。
Background: Heavy proteinuria in antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (GN) is usually considered to be associated with immune deposits in renal biopsy. Nephrotic ANCA GN without immune deposits (pauci-immune) is rare and has not been studied specially. In this study characteristics of these patients are to be investigated. Methods: Clinical and pathological characteristics from 20 kidney biopsy-proven pauci-immune anti-myeloperoxidase antibody-associated GN patients with nephrotic proteinuria were analyzed and were compared with ANCA GN patients without nephrotic proteinuria. Results: Acute kidney injury (AKI) and gross hematuria were much prevalent but extra-renal involvement was less prevalent in pauci-immune ANCA GN with nephrotic proteinuria than in pauci-immune ANCA GN without nephrotic proteinuria. No more severe hypoalbuminemia, hypercoagulability, hyperlipidemia or higher thrombosis incidence were found between two groups. Compared with patients without nephrotic proteinuria, patients with nephrotic proteinuria had more prevalent crescentic category in histopathology. Proteinuria decreased quickly after treatment but much poorer renal prognosis was found in pauci-immune ANCA GN with nephrotic proteinuria. The results of urinary albumin to total protein ratio and urinary protein electrophoresis showed pauci-immune ANCA GN with nephrotic proteinuria had obvious non-selective proteinuria. Conclusions: Pauci-immune ANCA GN with nephrotic proteinuria do not have more severe hypoalbuminemia, hypercoagulability or hyperlipidemia than patients without nephrotic proteinuria. Non-selective proteinuria might be the reason. However, pauci-immune ANCA GN with nephrotic proteinuria have more prevalent crescentic category in histopathology, higher incidence of AKI, gross hematuria and poorer renal prognosis despite of good sensitivity to therapy of proteinuria.
影响因子：
0.8
作者：
Singh NP;Gulati S;Garg V;Beniwal P;Garg S
通讯作者：
Garg S