特发性膜性肾病伴局灶性节段硬化

Pub Date : 2024-07-07 DOI:10.26442/00403660.2024.06.202725
E. Kamyshova, I. N. Bobkova, P. Kakhsurueva, Amnat S. Abdulaeva, T. Rudenko, E. Stavrovskaya, E. Y. Andreeva, O.A. Li, A. Suvorov
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引用次数: 0

摘要

目的评估一组俄罗斯患者特发性膜性肾病(IMN)伴局灶节段性硬化(FSGS)的临床和病理特征及预后。材料与方法我们的单中心队列回顾性研究共纳入了 101 名经形态学验证的特发性膜性肾病患者。患者被分为 IMN 组和 IMN+FSGS 组。对随访数据超过 6 个月的 59 名患者的主要和次要结果进行了分析。研究结果肾活检时的中位年龄为 46.0 (33.0; 55.0) 岁,中位随访时间为 6.8 (4.0; 15.6) 个月。15例(14.9%)IMN患者出现继发性FSGS。IMN 组和 IMN+FSGS 组在性别、IMN 发病年龄和肾活检年龄方面没有差异。与 IMN 组相比,IMN+FSGS 组的蛋白尿更高,估计肾小球滤过率更低(P0.05)。IMN+FSGS 组的收缩压和肌酐水平略高于 IMN 组,但差异不显著。两组的抗 PLA2R 阳性率相似。在 IMN 组和 IMN+FSGS 组中,分别有 10/52 (19.2%)和 5/7(71.4%)名患者观察到慢性肾病(CKD)进展。在多变量 Cox 回归模型中,肾活检年龄(几率比 - OR 1.12,95% 置信区间 - CI 1.03-1.22;р=0.07)、FSGS(OR 0.05,95% CI 0.01-0.34;р=0.002)和对初始免疫抑制疗程的反应(OR 0.33,95% CI 0.12-0.95;р=0.039)与 CKD 进展相关。结论在 IMN 患者中,继发性 FSGS 与更严重的蛋白尿和估计肾小球滤过率下降有关,也是 CKD 进展的一个独立因素。
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Idiopathic membranous nephropathy with focal segmental sclerosis
Aim. To evaluate the clinical and pathological features and prognosis of idiopathic membranous nephropathy (IMN) with focal segmental sclerosis (FSGS) in a group of Russian patients. Materials and methods. 101 patients with morphologically verified IMN were enrolled in our single-center cohort retrospective study. The patients were divided into IMN group and IMN+FSGS group. The primary and secondary outcomes were analyzed in 59 patients, which had follow-up data for period more than 6 months. Results. At the time of renal biopsy the median age was 46.0 (33.0; 55.0) years and the median follow-up was 6.8 (4.0; 15.6) months. Secondary FSGS was revealed in 15 (14.9%) patients with IMN. The IMN and IMN+FSGS groups did not differ in gender, age of onset IMN and age of renal biopsy. In the IMN+FSGS group proteinuria was higher and estimated glomerular filtration rate was lower than that in the IMN group (p0.05). The systolic arterial pressure and creatinine levels in the IMN+FSGS group were slightly higher than in the IMN group, but the difference was not significant. Anti-PLA2R positivity was similar in both groups. Chronic kidney disease (CKD) progression was observed in 10/52 (19.2%) and 5/7 (71.4%) patients in IMN and IMN+FSGS groups, respectively. In a multivariate Cox regression model, age of renal biopsy (odds ratio – OR 1.12, 95% confidence interval – CI 1.03–1.22; р=0.07), FSGS (OR 0.05, 95% CI 0.01–0.34; р=0.002) и response to initial course of immunosuppression (OR 0.33, 95% CI 0.12–0.95; р=0.039) were associated with the CKD progression. Conclusion. In patients with IMN secondary FSGS is associated with a greater severity of proteinuria and a decrease in estimated glomerular filtration rate, and is also an independent factor of the CKD progression.
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