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儿童免疫球蛋白A肾病糖皮质激素治疗的回顾性分析:肾脏结局与疗效

Retrospective analysis of glucocorticoid therapy in pediatric immunoglobulin A nephropathy: Kidney outcomes and efficacy.

作者信息

Wu Heyan, Xia Zhengkun, Zhang Lidan

机构信息

Pediatric Intensive Care Unit, The Seventh Affiliated Hospital of Sun Yat-sen University, 628 Zhenyuan Road, Guangming District, Shenzhen, 518107, Guangdong Province, China.

Department of Pediatrics, Jinling Hospital, 305 East Zhongshan Road, Nanjing, 210002, Jiangsu Province, China.

出版信息

Pediatr Nephrol. 2025 Jul 15. doi: 10.1007/s00467-025-06845-8.

DOI:10.1007/s00467-025-06845-8
PMID:40663186
Abstract

BACKGROUND

The efficacy of glucocorticoid (GC) in the management of immunoglobulin A nephropathy (IgAN) remains highly controversial. The study was conducted to analyze the efficacy and kidney outcomes of GC in the treatment of pediatric IgAN.

METHODS

Using the follow-up data of children with chronic kidney disease from the Department of Pediatrics at Jinling Hospital between January 2000 and December 2020, we selected children with primary IgAN who were ≤ 18 years old, confirmed by kidney biopsy, and had undergone regular follow-up for more than 2 years. Patients who had previously used other immunosuppressive agents or had not received renin-angiotensin system blocker (RASB) treatment were excluded. The selected patients were divided into two groups based on their prior treatment regimens: the GC + RASB group and the RASB group. The primary outcome was a composite of a 40% decrease in estimated glomerular filtration rate (eGFR) from baseline, kidney failure, or death due to kidney disease.

RESULTS

A total of 374 patients (149 females) were enrolled, with 230 in the GC + RASB group and 144 in the RASB group. At baseline, the GC + RASB group had lower albumin and higher creatinine levels (all P < 0.05). From 6 months of treatment, the GC + RASB group showed higher urinary protein remission rates (P < 0.05), but hematuria relief was similar between groups. Adverse events, including centripetal obesity, were more frequent in the GC + RASB group (P = 0.001). After a median follow-up of 130.97 months, the GC + RASB group had fewer endpoint events (5.22% vs. 11.11%, P = 0.035) and higher cumulative kidney event-free survival rates, particularly in patients with eGFR > 50 ml/min/1.73 m and 24 h-UP ≥ 1 g/d (all P < 0.05).

CONCLUSIONS

GC therapy reduced the risk of progression to kidney failure in children with initial eGFR > 50 ml/min/1.73 m and proteinuria ≥ 1 g/d. No additional kidney event-free survival benefit was observed in children with eGFR ≤ 50 ml/min/1.73 m or proteinuria < 1 g/d.

摘要

背景

糖皮质激素(GC)在免疫球蛋白A肾病(IgAN)治疗中的疗效仍存在高度争议。本研究旨在分析GC治疗儿童IgAN的疗效及肾脏转归。

方法

利用2000年1月至2020年12月期间金陵医院儿科慢性肾脏病患儿的随访数据,选取年龄≤18岁、经肾活检确诊且接受规律随访超过2年的原发性IgAN患儿。排除既往使用过其他免疫抑制剂或未接受肾素 - 血管紧张素系统阻滞剂(RASB)治疗的患者。根据既往治疗方案将入选患者分为两组:GC + RASB组和RASB组。主要结局为估计肾小球滤过率(eGFR)较基线下降40%、肾衰竭或因肾脏疾病死亡的复合结局。

结果

共纳入374例患者(149例女性),GC + RASB组230例,RASB组144例。基线时,GC + RASB组白蛋白水平较低,肌酐水平较高(均P < 0.05)。治疗6个月起,GC + RASB组尿蛋白缓解率较高(P < 0.05),但两组血尿缓解情况相似。包括向心性肥胖在内的不良事件在GC + RASB组更常见(P = 0.001)。中位随访130.97个月后,GC + RASB组终点事件较少(5.22% 对11.11%,P = 0.035),累积肾脏无事件生存率较高,尤其是初始eGFR > 50 ml/min/1.73 m²且24小时尿蛋白(24 h-UP)≥1 g/d的患者(均P < 0.05)。

结论

GC治疗可降低初始eGFR > 50 ml/min/1.73 m²且蛋白尿≥1 g/d的儿童进展至肾衰竭的风险。在eGFR≤50 ml/min/1.73 m²或蛋白尿<1 g/d的儿童中未观察到额外的肾脏无事件生存获益。

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