Cure Glomerulonephropathy Pathology Classification and Core Scoring Criteria, Reproducibility, and Clinicopathologic Correlations.
Cure Glomerulonephropathy Pathology Classification and Core Scoring Criteria, Reproducibility, and Clinicopathologic Correlations.
复制标题
DOI：
10.1159/000534755
复制
发表时间：
2023-01
期刊：
影响因子：
--
通讯作者：
中科院分区：
文献类型：
--
作者：
Cure肾小球肾病（CureGN）是一项在微小病变疾病（MCD）、局灶节段性肾小球硬化（FSGS）、膜性肾病（MN）或伊加肾病患者中开展的观察性队列研究。我们开发了一个传统的，基于共识的评分系统，以记录病理学特征的应用程序在多个病理学家，并在此描述的协议，再现性和相关性与临床参数活检。定义肾小球，肾小管，间质和血管病变的半定量评价使用数字化光学显微镜载玻片和电子显微镜，并报告免疫荧光。截至2019年4月，由随机分配的病理学家对具有策划病理材料的病例进行评分，其中至少10%的病例由第二位病理学家进行评分。使用Gwet一致系数（AC）1统计量评估评分再现性，并与临床变量进行相关性分析。在800份评分活检（134份MCD、194份FSGS、206份MN、266份伊加）中，94份评分两次（11.8%）。在60个病理学特征中，46个（76.7%）表现为极好（AC 1>0.8），12个（20.0%）表现为良好（AC 1 0.6-0.8）。肾小球系膜细胞增生评分为不存在、局灶性或弥漫性时具有中等重现性（AC 1 = 0.58），但评分为不存在或局灶性与弥漫性时具有良好重现性（AC 1 = 0.71）。肾小球百分比评分为无病变，具有良好的再现性（AC 1 = 0.34）。病理特征与活检时临床特征之间最强的相关性包括间质性炎症、间质性纤维化和肾小管萎缩与估计的肾小球滤过率、足突消失与尿蛋白/肌酐比值以及活动性新月体与血尿。大多数评分的病理学特征显示出良好的再现性，证明了多位病理学家对这些特征的一致性。某些病理特征和预期的临床特征之间的相关性显示了这种方法对于未来临床病理相关性和生物标志物发现的研究的价值。
Cure Glomerulonephropathy (CureGN) is an observational cohort study of patients with minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), membranous nephropathy (MN), or IgA nephropathy. We developed a conventional, consensus-based scoring system to document pathologic features for application across multiple pathologists and herein describe the protocol, reproducibility, and correlation with clinical parameters at biopsy. Definitions were established for glomerular, tubular, interstitial, and vascular lesions evaluated semiquantitatively using digitized light microscopy slides and electron micrographs, and reported immunofluorescence. Cases with curated pathology materials as of April 2019 were scored by a randomly assigned pathologist, with at least 10% of cases scored by a second pathologist. Scoring reproducibility was assessed using Gwet’s agreement coefficient (AC)1 statistic and correlations with clinical variables were performed. Of 800 scored biopsies (134 MCD, 194 FSGS, 206 MN, 266 IgA), 94 were scored twice (11.8%). Of 60 pathology features, 46 (76.7%) demonstrated excellent (AC1>0.8), and 12 (20.0%) had good (AC1 0.6–0.8) reproducibility. Mesangial hypercellularity scored as absent, focal, or diffuse had moderate reproducibility (AC1 = 0.58), but good reproducibility (AC1 = 0.71) when scored as absent or focal versus diffuse. The percent glomeruli scored as no lesions had fair reproducibility (AC1 = 0.34). Strongest correlations between pathologic features and clinical characteristics at biopsy included interstitial inflammation, interstitial fibrosis, and tubular atrophy with estimated glomerular filtration rate, foot process effacement with urine protein/creatinine ratio, and active crescents with hematuria. Most scored pathology features showed excellent reproducibility, demonstrating consistency for these features across multiple pathologists. Correlations between certain pathologic features and expected clinical characteristics show the value of this approach for future studies on clinicopathologic correlations and biomarker discovery.