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抗磷脂酶 A2 受体阳性膜性肾病:探讨电子显微镜分期与临床结局的关系。

Anti-phospholipase A2 receptor positive membranous nephropathy: investigating the link between electron microscopy stages and clinical outcome.

机构信息

Nephrology Department, University of Medicine and Pharmacy "Carol Davila", Bucharest, Romania.

Nephrology Department, "Dr. Carol Davila" Teaching Hospital of Nephrology, Bucharest, Romania.

出版信息

Ultrastruct Pathol. 2023 Sep 3;47(5):365-372. doi: 10.1080/01913123.2023.2236225. Epub 2023 Jul 14.

Abstract

This retrospective, observational study sought to examine the relationship between Ehrenreich-Churg electron microscopy (EM) stages and long-term outcomes in anti-PLA2R membranous nephropathy (MN). Seventy-one patients with anti-PLA2R MN (median titer 185.7RU/mL) were followed for a median of 46 months, with end-stage kidney disease (ESKD) as the primary endpoint, and response to treatment as a secondary endpoint. Patients were grouped into stages I-II (41 patients) and stages III-IV (30 patients) for analytical purposes. Notably, the III-IV group demonstrated a lower eGFR, lower anti-PLA2R titer, but a higher chronicity score. Kaplan-Meier analysis showed shorter mean kidney survival time in stages III-IV compared to I-II (p 0.03). However, multivariate analysis using Cox regression indicated that Ehrenreich-Churg stages did not significantly influence kidney survival, but lower eGFR at diagnosis and higher histopathological chronicity score did. Remission was achieved by 64% of patients and no relationship between Ehrenreich-Churg stages and treatment response was found. The only identified risk factor for not achieving remission was the severity of hyposerinemia at diagnosis. In conclusion, while EM stages III-IV are associated with more chronic lesions and stages I-II with more active immunologic disease, the histological chronicity score seems to be a stronger predictor of long-term outcomes.

摘要

这项回顾性观察研究旨在探讨抗-PLA2R 膜性肾病(MN)中 Ehrenreich-Churg 电子显微镜(EM)分期与长期结局之间的关系。71 例抗-PLA2R MN 患者(中位滴度 185.7RU/mL)接受了中位随访 46 个月,以终末期肾病(ESKD)为主要终点,以治疗反应为次要终点。为分析目的,患者被分为 I-II 期(41 例)和 III-IV 期(30 例)。值得注意的是,III-IV 期患者的 eGFR 较低、抗-PLA2R 滴度较低,但慢性评分较高。Kaplan-Meier 分析显示,III-IV 期的平均肾脏生存时间短于 I-II 期(p=0.03)。然而,使用 Cox 回归的多变量分析表明,Ehrenreich-Churg 分期并未显著影响肾脏生存,但诊断时的 eGFR 较低和更高的组织病理学慢性评分确实有影响。64%的患者实现了缓解,且未发现 Ehrenreich-Churg 分期与治疗反应之间存在关系。未达到缓解的唯一确定风险因素是诊断时低血清素血症的严重程度。总之,虽然 EM 分期 III-IV 与更慢性病变有关,而 I-II 期与更活跃的免疫性疾病有关,但组织学慢性评分似乎是长期结局的更强预测因素。

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