Department of Pediatrics, Wakayama Medical University, Wakayama City, Wakayama, Japan.
Nephrol Dial Transplant. 2011 Jan;26(1):163-9. doi: 10.1093/ndt/gfq387. Epub 2010 Jul 2.
The prognosis of children with severe IgA nephropathy showing diffuse mesangial proliferation is poor. However, the prognosis can be improved by combination therapy (prednisolone + azathioprine or mizoribine + warfarin + dipyridamole) or prednisolone alone over a 2-year period, and disappearance of glomerular IgA deposits is often observed. Details of the incidence and clinicopathological significance of glomerular IgA disappearance remain unclear.
To investigate this phenomenon, we retrospectively screened and analysed 124 consecutive children (age ≤ 18 years at first biopsy) with newly diagnosed severe IgA nephropathy showing diffuse mesangial proliferation, who received combination therapy or prednisolone alone for 2 years and underwent repeat biopsies.
Among these patients, 90 received combination therapy, and 34 received prednisolone alone. After 2 years of treatment, 27 of the patients (21.8%) showed disappearance of glomerular IgA. Logistic analysis showed that IgA disappearance was associated with less severe urinary protein excretion at the end of treatment. Kaplan-Meier analysis of the long-term course revealed a significant difference in proteinuria-free survival after the 2-year treatment period between the patients with IgA disappearance and those without (P = 0.008; log-rank test). The Cox proportional hazards model showed that disappearance of glomerular IgA after the treatment was a factor significantly associated with proteinuria-free survival in both univariate and multivariate analyses.
The present results suggest that disappearance of IgA after 2 years of treatment indicates milder disease severity, even in patients with diffuse mesangial proliferation, and is a prognostic factor related to proteinuria-free survival.
表现为弥漫性系膜增殖的严重 IgA 肾病患儿的预后较差。然而,通过 2 年的联合治疗(泼尼松龙+硫唑嘌呤或霉酚酸酯+华法林+双嘧达莫)或泼尼松龙单独治疗,预后可以得到改善,并且常观察到肾小球 IgA 沉积的消失。然而,IgA 消失的发生率和临床病理意义的细节仍不清楚。
为了研究这一现象,我们回顾性筛选和分析了 124 例连续的新诊断为弥漫性系膜增殖的严重 IgA 肾病患儿(首次活检时年龄≤18 岁),这些患儿接受了 2 年的联合治疗或泼尼松龙单独治疗,并进行了重复活检。
其中 90 例接受联合治疗,34 例接受泼尼松龙单独治疗。治疗 2 年后,27 例患者(21.8%)出现肾小球 IgA 消失。逻辑分析表明,IgA 消失与治疗结束时尿蛋白排泄量较轻有关。Kaplan-Meier 分析长期病程显示,在 2 年治疗期后,IgA 消失患者与无 IgA 消失患者的蛋白尿无复发生存率存在显著差异(P=0.008;log-rank 检验)。Cox 比例风险模型显示,治疗后肾小球 IgA 消失是单因素和多因素分析中蛋白尿无复发生存的一个显著相关因素。
本研究结果表明,治疗 2 年后 IgA 的消失表明疾病严重程度较轻,即使在弥漫性系膜增殖的患者中也是如此,并且是与蛋白尿无复发生存相关的预后因素。