Fibrillary Glomerulonephritis: Clinicopathologic Features and Atypical Cases from a Multi-Institutional Cohort
Fibrillary Glomerulonephritis: Clinicopathologic Features and Atypical Cases from a Multi-Institutional Cohort
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DOI：
10.2215/cjn.03870319
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发表时间：
2019-12-06
影响因子：
9.8
通讯作者：
Smith, Kelly D.
中科院分区：
文献类型：
--
作者：
Andeen, Nicole K.;Troxell, Megan L.;Smith, Kelly D.
背景和目的纤维性肾炎被定义为一种免疫复合物介导的肾小球肾炎，其淀粉样纤维大于淀粉样蛋白，免疫球蛋白阳性，刚果红阴性。随着DNAJB9被发现为纤维性GN的高度敏感和特异的标志物，建立纤维性GN诊断的形态学标准的特异性受到了质疑。设计、背景、参与者和测量我们试图(1)确定最能定义纤维性GN的解剖学特征，(2)确定预测结果的临床和病理特征。结果我们回顾了1997至2017年间诊断为纤维性GN或可疑纤维性GN的患者(n=266，女性65%，中位年龄61岁)的肾活检。大约11%的肾活检有一个或多个不寻常的特征，包括单型沉积、刚果红阳性或不寻常的纤维直径。纤维性肾小球肾炎可能是一种具有肾脏意义的单克隆性伽马病
Background and objectivesFibrillary GN has been defined as an immune complex-mediated GN with amyloid-like fibrils larger than amyloid which are IgG positive and Congo red negative. With discovery of DNAJB9 as a highly sensitive and specific marker for fibrillary GN, the specificity of the morphologic criteria for establishing the diagnosis of fibrillary GN has come into question.Design, setting, participants, & measurementsWe sought to (1) determine anatomic characteristics that best define fibrillary GN and (2) identify clinical and pathologic features that predict outcomes.ResultsWe retrospectively reviewed kidney biopsies from patients diagnosed with fibrillary GN or suspected fibrillary GN between 1997 and 2017 (n=266, 65% female, median age 61). Approximately 11% of kidney biopsies had one or more unusual feature including monotypic deposits, Congo red positivity, or unusual fibril diameter. Fibrillary GN as a possible monoclonal gammopathy of renal significance represented