In Patients with Membranous Lupus Nephritis, Exostosin-Positivity and Exostosin-Negativity Represent Two Different Phenotypes
In Patients with Membranous Lupus Nephritis, Exostosin-Positivity and Exostosin-Negativity Represent Two Different Phenotypes
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DOI：
10.1681/asn.2020081181
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发表时间：
2021-03-01
影响因子：
13.6
通讯作者：
Sethi, Sanjeev
中科院分区：
文献类型：
--
作者：
Ravindran, Aishwarya;Moura, Marta Casal;Sethi, Sanjeev
在继发性（自身免疫性）膜性肾病患者中，两种新蛋白Exostosin 1和Exostosin 2（EXT 1/EXT 2）是潜在的疾病抗原、生物标志物或两者兼而有之。在这项研究中，我们验证了EXT 1/EXT 2的研究结果在一个大型队列的膜性狼疮肾炎。方法回顾性分析2010年1月至2011年12月间收治的膜性狼疮性肾炎患者的临床资料，并对肾活检标本进行抗EXT 1和EXT 2的免疫组化研究。比较了EXT 1/EXT 2阳性与EXT 1/EXT 2阴性患者的临床病理特征和结局。结果本组374例经活检证实的膜性狼疮性肾炎患者中，EXT 1/EXT 2阳性122例（32.6%），阴性252例（67.4%）。EXT 1/EXT 2阳性患者明显年轻（P=0.01），血清肌酐水平明显较低（P=0.02），蛋白尿>= 3.5 g/24 h的可能性明显更大（P=0.009），慢性特征明显较少（肾小球硬化，P=0.001或间质纤维化和肾小管萎缩，P
Background In patients with secondary (autoimmune) membranous nephropathy, two novel proteins, Exostosin 1 and Exostosin 2 (EXT1/EXT2), are potential disease antigens, biomarkers, or both. In this study, we validate the EXT1/EXT2 findings in a large cohort of membranous lupus nephritis. Methods We conducted a retrospective cohort study of patients with membranous lupus nephritis, and performed immunohistochemistry studies on the kidney biopsy specimens against EXT1 and EXT2. Clinicopathologic features and outcomes of EXT1/EXT2-positive versus EXT1/EXT2-negative patients were compared. Results Our study cohort included 374 biopsy-proven membranous lupus nephritis cases, of which 122 (32.6%) were EXT1/EXT2-positive and 252 (67.4%) were EXT1/EXT2-negative. EXT1/EXT2-positive patients were significantly younger (P=0.01), had significantly lower serum creatinine levels (P=0.02), were significantly more likely to present with proteinuria >= 3.5 g/24 h (P=0.009), and had significantly less chronicity features (glomerulosclerosis, P=0.001 or interstitial fibrosis and tubular atrophy, P