Clinical characteristics of juvenile systemic lupus erythematosus with hemophagocytic lymphohistiocytosis as the first symptom and literature review

WAN Lijun; WU Bei; YANG Yan; TAN Pei; ZHAO Alan; ZHAN Xiaoyun; LU Yinghong; HUANG Juan

doi:10.14188/j.1671-8852.2024.0472

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Clinical characteristics of juvenile systemic lupus erythematosus with hemophagocytic lymphohistiocytosis as the first symptom and literature review

更新时间：2026-11-05

- Clinical characteristics of juvenile systemic lupus erythematosus with hemophagocytic lymphohistiocytosis as the first symptom and literature review
- Medical Journal of Wuhan University Vol. 46, Issue 11, Pages: 1481-1487(2025)
- 作者机构：
  
  华中科技大学同济医学院附属湖北妇幼保健院儿童血液科湖北武汉 430070
- 作者简介：
- 基金信息：
- DOI：10.14188/j.1671-8852.2024.0472
  CLC： R725.5
- Published：25 November 2025
- 稿件说明：
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万丽君,武贝,杨琰等.以噬血细胞性淋巴组织细胞增多症为首发症状儿童系统性红斑狼疮的临床特点并文献复习[J].武汉大学学报（医学版）,2025,46(11):1481-1487.

WAN Lijun,WU Bei,YANG Yan,et al.Clinical characteristics of juvenile systemic lupus erythematosus with hemophagocytic lymphohistiocytosis as the first symptom and literature review[J].Medical Journal of Wuhan University,2025,46(11):1481-1487.
万丽君,武贝,杨琰等.以噬血细胞性淋巴组织细胞增多症为首发症状儿童系统性红斑狼疮的临床特点并文献复习[J].武汉大学学报（医学版）,2025,46(11):1481-1487. DOI： 10.14188/j.1671-8852.2024.0472.

WAN Lijun,WU Bei,YANG Yan,et al.Clinical characteristics of juvenile systemic lupus erythematosus with hemophagocytic lymphohistiocytosis as the first symptom and literature review[J].Medical Journal of Wuhan University,2025,46(11):1481-1487. DOI： 10.14188/j.1671-8852.2024.0472.

摘要

目的

通过查阅文献总结以噬血细胞性淋巴组织细胞增多症（HLH）为首发症状的儿童系统性红斑狼疮（JSLE）⁃巨噬细胞活化综合征（MAS）病例特点，并报道1例典型病例为临床诊疗提供参考。

方法

计算机检索PubMed、The Cochrane Library、CNKI、Web of Science和万方数据库，收集从建库至2024年5月1日发表的以HLH为首发症状的JSLE文献，总结其临床表现、实验室检查、治疗及临床转归，并报道1例典型案例。

结果

本文案例系国内报道第7例。以HLH为首发症状的JSLE均有发热及血两系或三系下降，其他临床表现异质性较高。实验室方面均有肝功能异常，乳酸脱氢酶显著升高和铁蛋白异常上升常见；自然杀伤（NK）细胞比例下降但活性下降不常见，可溶性白细胞介素⁃2受体α链（sCD25）异常者不常见；骨髓噬血现象常见；早期肾功能均正常，尿常规可见尿蛋白或潜血阳性；起病时抗核抗体（ANA）阳性率不高；治疗多予甲泼尼松龙冲击疗法，预后良好。

结论

以HLH起病的JSLE罕见，异质性高，对于持续发热，先达到HLH标准患儿应迅速启动原发病查找；同时应加大基因组学、表观组学、代谢组学等多组学研究，探明其发病机制。

Abstract

Objective

To review reported cases of juvenile systemic lupus erythematosus–macrophage activation syndrome (JSLE⁃MAS) initially presenting as hemophagocytic lymphohistiocytosis (HLH)

summarize their characteristics

and describe a typical JSLE⁃HLH case to inform clinical diagnosis and treatment.

Methods

A computerized search of PubMed

The Cochrane Library

CNKI

Web of Science and Wanfang databases was conducted to collect the literature on children with HLH as the first symptom of JSLE

which was published from the establishment of the library to May 2024

and we summarized its clinical manifestations

laboratory tests

treatment and clinical outcome. Then we reported a typical case of JSLE.

Results

JSLE with HLH as the initial symptom presented with fever and a decrease in two or three blood cell lines

while other clinical manifestations showed high heterogeneity. Laboratory findings included abnormal liver function

significantly elevated lactate dehydrogenase

and abnormally increased ferritin levels; decreased natural killer (NK) cell proportions but decreased activity was uncommon

and soluble interleukin⁃2 receptor alpha chain (sCD25) abnormalities were rare; bone marrow hemophagocytosis was common. Early renal function was normal

and urine routine tests showed proteinuria or positive occult blood; the positive rate of antinuclear antibody (ANA) at the onset was not high. Treatment mainly involved high⁃dose methylprednisolone shock therapy

with a good prognosis. This paper reported the 7th case in China.

Conclusion

JSLE with HLH as the initial symptom is rare and highly heterogeneous. For children with persistent fever who meet the HLH criteria

the search for the primary disease should be initiated promptly. At the same time

it is necessary to increase the study of genome

epigenomics

metabolomics and other omics to explore its pathogenesis.

关键词

Keywords

references

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