Kamyshova E S, Bobkova I N, Kakhsurueva P А, Abdulaeva A S, Rudenko T Е, Stavrovskaya E V, Andreeva E Y, Li O А, Suvorov A Y
Sechenov First Moscow State Medical University (Sechenov University).
Municipal Polyclinic No. 134.
Ter Arkh. 2024 Jul 7;96(6):580-586. doi: 10.26442/00403660.2024.06.202725.
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.
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.
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 (<0.05). 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; =0.07), FSGS (OR 0.05, 95% CI 0.01-0.34; =0.002) и response to initial course of immunosuppression (OR 0.33, 95% CI 0.12-0.95; =0.039) were associated with the CKD progression.
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.
评估一组俄罗斯患者中合并局灶节段性肾小球硬化(FSGS)的特发性膜性肾病(IMN)的临床、病理特征及预后。
101例经形态学证实的IMN患者纳入我们的单中心队列回顾性研究。患者分为IMN组和IMN+FSGS组。对59例有超过6个月随访数据的患者分析主要和次要结局。
肾活检时中位年龄为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患者发现继发性FSGS。IMN组和IMN+FSGS组在性别、IMN发病年龄和肾活检年龄方面无差异。IMN+FSGS组蛋白尿高于IMN组,估计肾小球滤过率低于IMN组(<0.05)。IMN+FSGS组收缩压和肌酐水平略高于IMN组,但差异无统计学意义。两组抗PLA2R阳性率相似。IMN组和IMN+FSGS组分别有10/52(19.2%)和5/7(71.4%)的患者出现慢性肾脏病(CKD)进展。在多变量Cox回归模型中,肾活检年龄(比值比-OR 1.12,95%置信区间-CI 1.03-1.22;P=0.07)、FSGS(OR 0.05,95%CI 0.01-0.34;P=0.002)及对初始免疫抑制疗程的反应(OR 0.33,95%CI 0.12-0.95;P=0.039)与CKD进展相关。
在IMN患者中,继发性FSGS与更严重的蛋白尿及估计肾小球滤过率降低相关,也是CKD进展的独立因素。