The clinicopathologic spectrum of segmental membranous glomerulopathy
The clinicopathologic spectrum of segmental membranous glomerulopathy
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DOI：
10.1016/j.kint.2020.06.014
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发表时间：
2021-01-01
影响因子：
19.6
通讯作者：
Markowitz, Glen S.
中科院分区：
文献类型：
--
作者：
Kudose, Satoru;Santoriello, Dominick;Markowitz, Glen S.
膜性肾小球病（MGN）的特点是全球性的上皮下免疫沉积，免疫荧光对IgG染色最强烈。在这里，我们描述了一组MGN患者的临床和病理结果，根据定义，只有部分免疫沉积存在。这种罕见的变异，被称为节段性MGN (sMGN)，其特征很差。我们回顾性分析了2010年1月至2018年10月在哥伦比亚大学诊断的所有sMGN患者，不包括系统性红斑狼疮患者。收集了表现特征、病理表现和结果的数据。确认了50例sMGN，占MGN的2.5%。在50例活组织检查中，有21例有另一种主要的疾病过程。其余29例分离性sMGN患者中位肌酐为0.97 mg/dl，中位24小时尿蛋白为3.1 g/天，32%有肾病综合征。17例中有5例NELL-1（一种蛋白激酶C结合蛋白）染色阳性。在所有活检评估中，PLA2R、THSD7A和exostosin 1（原发性MGN中的自身抗原）的染色均为阴性。超微结构检查主要显示早期sMGN（14/29的1期或1-2期）。29例分离性sMGN患者中有21例（中位12个月）进行了随访，包括7例接受免疫抑制（主要是糖皮质激素）的患者。在随访期间，86%的患者肾功能稳定/改善，45%的患者完全缓解，15%的患者部分缓解。在15例无完全肾病综合征的孤立性sMGN患者中，仅有2例接受了免疫抑制；尽管如此，50%的患者完全缓解，21%的患者部分缓解。因此，即使在没有免疫抑制治疗的情况下，sMGN也是一种罕见的pla2r阴性MGN变体，其NELL-1阳性率为29%，预后良好。
Membranous glomerulopathy (MGN) is characterized by global subepithelial immune deposits that stain most intensely by immunofluorescence for IgG. Here we describe the clinical and pathologic findings in a cohort of patients with MGN in which, by definition, only segmental immune deposits are present. This rare variant, termed segmental MGN (sMGN), is poorly characterized. We retrospectively identified all patients with sMGN diagnosed at Columbia University from January 2010 to October 2018, excluding those with systemic lupus erythematosus. Data on presenting features, pathologic findings, and outcomes were collected. Fifty cases of sMGN were identified, representing 2.5% of MGN. In 21 of 50 biopsies, there was an alternative, predominant disease process. The remaining 29 patients with isolated sMGN had a median creatinine of 0.97 mg/dl, median 24-hour urine protein 3.1 g/day, and 32% had nephrotic syndrome. Staining for NELL-1 (a protein kinase C binding protein) was positive in five of 17 cases. Staining for PLA2R, THSD7A, and exostosin 1 (autoantigens in primary MGN) was negative in all biopsies evaluated. Ultrastructural evaluation revealed predominantly early stage sMGN (stage 1 or 1-2 in 14/29). Follow-up was available for 21 of the 29 patients with isolated sMGN (median 12 months), including seven who received immunosuppression (primarily glucocorticoids). During follow-up, 86% had stable/improved kidney function and 45% achieved complete while 15% achieved partial remission. Among the 15 patients with isolated sMGN without full nephrotic syndrome, only two received immunosuppression; nonetheless, 50% achieved complete while 21% achieved partial remission. Thus, sMGN is a rare PLA2R-negative variant of MGN with 29% NELL-1 positivity and favorable prognosis, even in the absence of immunosuppressive treatment.