Nephrology Department, University of Medicine and Pharmacy "Carol Davila", Bucharest, Romania.
Nephrology Department, "Dr. Carol Davila" Teaching Hospital of Nephrology, Bucharest, Romania.
Ultrastruct Pathol. 2023 Sep 3;47(5):365-372. doi: 10.1080/01913123.2023.2236225. Epub 2023 Jul 14.
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.
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。