Department of Nephrology, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiaotong University, Shanghai, China.
BMC Pediatr. 2023 Jul 26;23(1):377. doi: 10.1186/s12887-023-04195-8.
There is no standard recommendation for IgA nephropathy treatment in children.
This is a retrospective study. From 2012 to 2020, newly diagnosed primary IgAN followed up for at least 1 year were enrolled. The correlation of MESTC scores and clinical index including proteinuria, gross hematuria and renal dysfunction was analyzed. Treatment and clinical response of 6 month, 1year and 3 year at follow up were also analyzed. Complete renal remission was calculated with Kaplan-Meier analysis.
The median follow up was 36 months, from 12 months to 87months in 40 IgAN children. Angiotensin-converting enzyme inhibitor (ACEI) was applied to all patients. 30% received ACEI alone; 15% received glucocorticoids; 37.5% received glucocorticoids plus cyclophosphamide, 17.5% received glucocorticoids plus mycophenolate mofetil. Individuals with diffuse mesangial hypercellularity (M1) were more likely to have nephrotic range proteinuria compared to patients with M0 (80% vs. 20%, P < 0.01). Complete renal remission at 6-month, 1-year and 3-year follow up is 50.25%, 70% and 87.5% respectively. Five-year complete renal remission calculated by Kaplan-Meier analysis is 58.4%. Although without significant difference, there is trend of better survival with complete renal remission in group of nephrotic range proteinuria onset. There is no severe adverse effect.
This study supports the use of glucocorticoids plus immunosuppressive in addition to ACEI in IgA nephrology pediatric patients with proteinuria. We suggest proactive immunosuppressive treatment in IgA nephropathy in children. This is from a single center in China as may not same results in other population.
目前对于儿童 IgA 肾病尚缺乏标准的治疗推荐。
本研究为回顾性研究,纳入了 2012 年至 2020 年间新诊断的原发性 IgA 肾病患者,随访时间至少 1 年。分析 MESTC 评分与蛋白尿、肉眼血尿和肾功能不全等临床指标的相关性,分析患者在随访 6 个月、1 年和 3 年时的治疗及临床应答情况,采用 Kaplan-Meier 法计算完全肾脏缓解率。
40 例 IgA 肾病患儿的中位随访时间为 36 个月(12 个月至 87 个月),所有患者均接受血管紧张素转换酶抑制剂(ACEI)治疗,30%单独应用 ACEI,15%应用糖皮质激素,37.5%应用糖皮质激素联合环磷酰胺,17.5%应用糖皮质激素联合吗替麦考酚酯。弥漫性系膜细胞增生(M1)患者更易出现肾病范围蛋白尿,而非 M0 患者(80%比 20%,P<0.01)。6 个月、1 年和 3 年时完全肾脏缓解率分别为 50.25%、70%和 87.5%。Kaplan-Meier 法计算的 5 年完全肾脏缓解率为 58.4%。尽管差异无统计学意义,但完全肾脏缓解组患者的肾病范围蛋白尿起始患者的生存情况有改善趋势。无严重不良事件发生。
本研究支持在 IgA 肾病患儿中除应用 ACEI 外,还可加用糖皮质激素和免疫抑制剂。我们建议在儿童 IgA 肾病中积极进行免疫抑制治疗。本研究来自中国的单中心研究,可能与其他人群的结果不同。