背景:膜增殖性肾小球肾炎(MPGN)可分为免疫复合物MPGN(IC-MPGN)和C3肾小球病(C3G),其中包括致密沉积病(DDD)和C3肾小球肾炎(C3GN)。这些病症由不同补体途径中的异常引起,并且可能导致不同的预后。然而,描述各自临床课程的研究有限。
方法:在本研究中,根据2002年2月至2022年12月进行的肾脏活检诊断为MPGN的日本儿科患者被重新分类为患有IC-MPGN或C3G(DDD或C3GN)。我们回顾性分析了这些患者的临床特征和预后。
结果:在25例MPGN患者中,3人(12.0%)被诊断为DDD,20(80.0%),C3GN,和两个(8.0%)带有IC-MPGN。有13例(65.0%)患者和1例(33.3%)患者在C3GN和DDD治疗后缓解,分别,无IC-MPGN患者获得缓解。中位随访期为5.3(2.5-8.9)年,两组患者均未进展至估计肾小球滤过率<15ml/min/1.73m2.出现轻度至中度蛋白尿的C3GN患者(n=8)单独接受肾素-血管紧张素系统抑制剂(RAS-I),在最后一次随访中,这些患者的尿蛋白肌酐比率显着降低,血清C3水平显着升高。
结论:大多数MPGN患者被诊断为C3GN。C3GN的缓解率很高,在大约5年的随访期间,没有患者出现肾衰竭。此外,C3GN伴轻度至中度蛋白尿的患者在单独使用RAS-I治疗时具有良好的预后,但持续的警惕对于确定长期预后是必要的.
BACKGROUND: Membranoproliferative glomerulonephritis (MPGN) can be divided into immune-complex MPGN (IC-MPGN) and C3 glomerulopathy (C3G), which includes dense deposit disease (DDD) and C3 glomerulonephritis (C3GN). These conditions result from abnormalities in different complement pathways and may lead to different prognoses. However, there are limited studies describing the respective clinical courses.
METHODS: In this study, Japanese pediatric patients diagnosed with MPGN based on kidney biopsies conducted between February 2002 and December 2022 were reclassified as having IC-MPGN or C3G (DDD or C3GN). We retrospectively analyzed the clinical characteristics and outcomes of these patients.
RESULTS: Out of 25 patients with MPGN, three (12.0%) were diagnosed with DDD, 20 (80.0%) with C3GN, and two (8.0%) with IC-MPGN. There were 13 (65.0%) patients and one (33.3%) patient in remission after treatment for C3GN and DDD, respectively, and no patients with IC-MPGN achieved remission. The median follow-up period was 5.3 (2.5-8.9) years, and none of the patients in either group progressed to an estimated glomerular filtration rate < 15 ml/min/1.73 m2. Patients with C3GN presenting mild to moderate proteinuria (n = 8) received a renin-angiotensin system inhibitor (RAS-I) alone, and these patients exhibited a significant decrease in the urinary protein creatinine ratio and a notable increase in serum C3 levels at the last follow-up.
CONCLUSIONS: Most patients with MPGN were diagnosed with C3GN. The remission rate for C3GN was high, and no patients developed kidney failure during the approximately 5-year follow-up. Additionally, patients with C3GN with mild to moderate proteinuria had good outcomes with RAS-I alone, but continued vigilance is necessary to determine long-term prognosis.