Membranous nephropathy: a single disease or a pattern of injury resulting from different diseases.
Membranous nephropathy: a single disease or a pattern of injury resulting from different diseases.
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DOI：
10.1093/ckj/sfab069
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发表时间：
2021-10
影响因子：
4.6
通讯作者：
Sethi S
中科院分区：
文献类型：
--
作者：
Sethi S
膜性肾病（MN）是一种以免疫复合物上皮下（SE）沉积导致肾小球基底膜增厚为特征的疾病。当没有疾病关联时，它通常被分类为原发性MN（70%），当存在潜在疾病关联（如狼疮，恶性肿瘤，感染或药物）时，则为继发性MN（30%）。磷脂酶A2受体（PLA 2 R）和含血小板反应蛋白1型结构域的7A（THSD 7A）分别是70%和1-5%的原发性MN的靶抗原。其余MN中的抗原未知。最近，在MN中已经鉴定出多种新的蛋白质/靶抗原。这些包括exostosin 1/2，神经表皮生长样蛋白1，semaphorin 3B，原钙粘蛋白7和神经细胞粘附分子1。这些抗原中的一些存在于原发性MN的背景中，一些存在于继发性MN中，一些存在于两者中，因此模糊了原发性和继发性MN之间的界限。初步研究表明，每种新的抗原相关MN都有不同的临床、肾活检结果和结局数据。我们认为，每一个新的蛋白/抗原相关的MN是一种特定的疾病，在常见的MN损伤模式的特点是增厚肾小球基底膜（GBM）与或不与光镜下的尖峰或针孔，颗粒免疫球蛋白G与或不与补体3的免疫荧光显微镜和SE电子致密沉积物的电子显微镜。换句话说，MN实际上只是由特定疾病引起的损伤模式，这些疾病导致SE免疫沉积物沿着GBM沉积。确定引起MN模式的特定疾病实体不仅对于精确诊断和管理，而且对于这些新描述的疾病的未来研究至关重要。
Membranous nephropathy (MN) is defined as disease entity characterized by thickening of the glomerular basement membranes due to subepithelial (SE) deposition of immune complexes. It is typically classified into primary MN (70%) when there is no disease association, and secondary MN (30%) when there is an underlying disease association such as lupus, malignancy, infections or drugs. Phospholipase A2 receptor (PLA2R) and thrombospondin type-1 domain-containing 7A (THSD7A) are target antigens in 70% and 1–5% of primary MN, respectively. The antigens in the remaining MN were not known. Recently, multiple novel proteins/target antigens have been identified in MN. These include exostosin 1/2, neural epidermal growth-like 1 protein, semaphorin 3B, protocadherin 7 and neural cell adhesion molecule 1. Some of these antigens are present in the setting of primary MN, some in secondary MN and some in both, thus blurring the lines between primary and secondary MN. Preliminary studies show that each of the new antigen-associated MN has distinct clinical, kidney biopsy findings and outcome data. We propose that each new protein/antigen-associated MN is a specific disease that results in the common MN pattern of injury characterized by thickened glomerular basement membrane (GBM) with or without spikes or pinholes on light microscopy, granular immunoglobulin G with or without complement 3 on immunofluorescence microscopy and SE electron-dense deposits on electron microscopy. In other words, MN is truly only a pattern of injury resulting from specific diseases that cause deposition of SE immune deposits along the GBM. It is of paramount importance to ascertain the specific disease entity causing the MN pattern not only for precise diagnosis and management, but also for future studies on these newly described diseases.
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