Subepithelial deposits with microspherular structures in membranous glomerulonephritis

IF 1.1 4区 医学 Q4 MICROSCOPY
H. Choung, J. Jean-Gilles, B. Goldman
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引用次数: 1

Abstract

ABSTRACT Rare cases of membranous glomerulopathy (MGN) with subepithelial deposits consisting of microspherular structures identified by electron microscopy have been described in the literature as either MGN with spherules or podocyte infolding glomerulopathy (PIG). The paucity of available studies shows a strong association with underlying autoimmune disease. To further understand the significance of subepithelial microspherular deposits, we retrospectively identified native kidney biopsies from 10 patients diagnosed as MGN with subepithelial microspherular structures identified by ultrastructural examination at the University of Rochester Medical Center (URMC) during an 11-year period. The majority were Caucasian (80%) with a mean age of 51.3 (±12.9) years. 50% had an autoimmune disorder, of which 80% were SLE. Two SLE cases had concomitant rheumatoid arthritis and Sjogren’s syndrome. One additional case had antiphospholipid syndrome and showed lupus-like features on biopsy. 40% were idiopathic and negative for PLA2R, NELL1, and THSD7A. MGN with subepithelial microspherular structures is frequently associated with an underlying autoimmune disease. The majority are negative for markers of primary MGN (PLA2R, THSD7A, and NELL1) and show features suggestive of secondary MGN.
膜性肾小球肾炎中具有微球结构的上皮下沉积
罕见的膜性肾小球病(MGN)的上皮下沉积由微球结构组成,在电子显微镜下被描述为带有小球体的MGN或足细胞内折叠肾小球病(PIG)。现有研究的缺乏表明与潜在的自身免疫性疾病有很强的联系。为了进一步了解上皮下微球沉积的意义,我们回顾性分析了罗切斯特大学医学中心(URMC) 11年间10例经超微结构检查诊断为MGN的肾活检患者的上皮下微球结构。多数为白种人(80%),平均年龄51.3(±12.9)岁。50%患有自身免疫性疾病,其中80%为SLE。2例SLE合并类风湿关节炎和干燥综合征。另外一例有抗磷脂综合征,活检显示狼疮样特征。40%为特发性,PLA2R、NELL1和THSD7A阴性。具有上皮下微球结构的MGN通常与潜在的自身免疫性疾病相关。大多数原发性MGN标记物(PLA2R、THSD7A和NELL1)阴性,显示继发性MGN的特征。
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来源期刊
Ultrastructural Pathology
Ultrastructural Pathology 医学-病理学
CiteScore
2.00
自引率
10.00%
发文量
40
审稿时长
6-12 weeks
期刊介绍: Ultrastructural Pathology is the official journal of the Society for Ultrastructural Pathology. Published bimonthly, we are the only journal to be devoted entirely to diagnostic ultrastructural pathology. Ultrastructural Pathology is the ideal journal to publish high-quality research on the following topics: Advances in the uses of electron microscopic and immunohistochemical techniques Correlations of ultrastructural data with light microscopy, histochemistry, immunohistochemistry, biochemistry, cell and tissue culturing, and electron probe analysis Important new, investigative, clinical, and diagnostic EM methods.
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