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菲律宾儿童期起病的狼疮性肾炎疾病进展和死亡率的预后因素:在一家三级医院进行的回顾性队列研究

Prognostic factors for disease progression and mortality of childhood-onset lupus nephritis in the Philippines: a retrospective cohort study in a tertiary hospital.

作者信息

Asis Carla Marie L, Tee Cherica A, Resontoc Lourdes Paula R

机构信息

Division of Pediatric Rheumatology, Department of Pediatrics, Philippine General Hospital - University of the Philippines Manila, City of Manila, Philippines.

Division of Pediatric Nephrology, Department of Pediatrics, Philippine General Hospital - University of the Philippines Manila, City of Manila, Philippines.

出版信息

Pediatr Rheumatol Online J. 2025 Aug 2;23(1):86. doi: 10.1186/s12969-025-01128-w.

DOI:10.1186/s12969-025-01128-w
PMID:40753228
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12318420/
Abstract

BACKGROUND

Lupus nephritis (LN) is a significant cause of morbidity and mortality in pediatric systemic lupus erythematosus (SLE), with more severe disease seen in childhood-onset cases. In low-to-middle-income countries like the Philippines, financial barriers and healthcare limitations exacerbate poor outcomes. This study aims to describe the clinical features, prognostic factors, and outcomes of childhood-onset LN at the Philippine General Hospital.

METHODS

A retrospective cohort study was conducted on patients under 19 years diagnosed with LN from January 2014 to December 2021. Clinical, laboratory, treatment, and outcome data were extracted from medical records. Survival was estimated using the Kaplan-Meier method. Cox proportional hazards regression and logistic regression were used to identify predictors of mortality and progression to chronic kidney disease 5 (CKD 5), respectively.

RESULTS

A total of 128 patients were included, with a mean age of 14.4 years at LN diagnosis and a female-to-male ratio of 13.2:1. Only 16% underwent kidney biopsy, mainly due to financial constraints. Treatment delays and poor adherence occurred in 60% and 38% of patients, respectively, largely due to financial hardship and limited healthcare access. The median follow-up was 2.2 years (range, 0.0-8.8 years). Nephrotic range proteinuria (hazard ratio [HR] 2.91), hypertension at diagnosis (odds ratio [OR] 5.57), and failure to achieve early partial response or complete remission (HR 3.69) were significant predictors of poor health outcomes. Twenty five patients (19.5%) died during the observation period with infection as the leading cause of mortality.

CONCLUSIONS

Childhood-onset LN remains associated with high morbidity and mortality in the Philippines, highlighting the urgent need for early diagnosis, expanded diagnostic access, infection risk mitigation, early therapeutic response monitoring, and strategies to enhance treatment adherence to improve outcomes.

摘要

背景

狼疮性肾炎(LN)是儿童系统性红斑狼疮(SLE)发病和死亡的重要原因,儿童期发病的病例病情往往更严重。在菲律宾等中低收入国家,经济障碍和医疗保健限制使不良后果更加恶化。本研究旨在描述菲律宾总医院儿童期发病的LN的临床特征、预后因素及结局。

方法

对2014年1月至2021年12月期间诊断为LN的19岁以下患者进行回顾性队列研究。从病历中提取临床、实验室、治疗及结局数据。采用Kaplan-Meier法估计生存率。分别使用Cox比例风险回归和逻辑回归来确定死亡率和进展为慢性肾脏病5期(CKD 5)的预测因素。

结果

共纳入128例患者,LN诊断时的平均年龄为14.4岁,女性与男性比例为13.2:1。仅16%的患者接受了肾活检,主要原因是经济限制。分别有60%和38%的患者出现治疗延迟和依从性差的情况,主要原因是经济困难和医疗服务可及性有限。中位随访时间为2.2年(范围:0.0 - 8.8年)。肾病范围蛋白尿(风险比[HR] 2.91)、诊断时高血压(比值比[OR] 5.57)以及未能实现早期部分缓解或完全缓解(HR 3.69)是健康结局不良的显著预测因素。25例患者(19.5%)在观察期内死亡,感染是主要死因。

结论

在菲律宾,儿童期发病的LN仍然与高发病率和高死亡率相关,这凸显了迫切需要早期诊断、扩大诊断途径、降低感染风险、监测早期治疗反应以及采取提高治疗依从性的策略以改善结局。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cdce/12318420/8d1b8fa8f803/12969_2025_1128_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cdce/12318420/8d1b8fa8f803/12969_2025_1128_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cdce/12318420/8d1b8fa8f803/12969_2025_1128_Fig1_HTML.jpg

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