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发热、惊厥与皮疹：一例伴巨噬细胞活化综合征的新生儿红斑狼疮病例报告

Fever, convulsions, and rash: a case report of neonatal lupus erythematosus with macrophage activation syndrome.

作者信息

Yu Jing, Xue Guohui, Liu Xiaohui, Zhan Chunlei, Huang Jinshi

机构信息

Department of Intensive Care, Jiangxi Children's Hospital, Nanchang, Jiangxi, China.

Department of Clinical Laboratory, Jiujiang City Key Laboratory of Cell Therapy, Jiujiang NO.1 People's Hospital, Jiujiang, Jiangxi, China.

出版信息

Front Pediatr. 2024 Aug 20;12:1381493. doi: 10.3389/fped.2024.1381493. eCollection 2024.

DOI:10.3389/fped.2024.1381493
PMID:39228436
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11368741/
Abstract

Neonatal lupus erythematosus (NLE) is a rare acquired autoimmune disease associated with the entry of maternal antibodies into the fetal circulation via the placenta during pregnancy. Macrophage activation syndrome (MAS) is a severe hyperinflammatory disease. Herein, we present a case of NLE with MAS accompanied by fever, convulsions, and rash. High-dose gamma globulin and non-shock doses of steroids can be used as a first-line treatment for NLE with MAS. Fever can be a clinical manifestation of NLE, especially cutaneous lupus. Rash recession could be used to judge whether the disease is effectively controlled by treatment.

摘要

新生儿红斑狼疮（NLE）是一种罕见的获得性自身免疫性疾病，与孕期母体抗体经胎盘进入胎儿循环有关。巨噬细胞活化综合征（MAS）是一种严重的高炎症性疾病。在此，我们报告一例伴有发热、惊厥和皮疹的NLE合并MAS病例。大剂量丙种球蛋白和非冲击剂量的类固醇可作为NLE合并MAS的一线治疗方法。发热可能是NLE的临床表现，尤其是皮肤型狼疮。皮疹消退可用于判断疾病是否通过治疗得到有效控制。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad32/11368741/9e56b676fee9/fped-12-1381493-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad32/11368741/03c53fd23a63/fped-12-1381493-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad32/11368741/08c3a702a12b/fped-12-1381493-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad32/11368741/86fa8c928268/fped-12-1381493-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad32/11368741/9e56b676fee9/fped-12-1381493-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad32/11368741/03c53fd23a63/fped-12-1381493-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad32/11368741/08c3a702a12b/fped-12-1381493-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad32/11368741/86fa8c928268/fped-12-1381493-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad32/11368741/9e56b676fee9/fped-12-1381493-g004.jpg

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本文引用的文献

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Central nervous system involvement and thrombocytopenia as predictors of mortality in children with hemophagocytic lymphohistiocytosis.中枢神经系统受累和血小板减少作为噬血细胞性淋巴组织细胞增生症患儿死亡率的预测因素。
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Macrophage activation syndrome in a newborn: report of a case associated with neonatal lupus erythematosus and a summary of the literature.新生儿巨噬细胞活化综合征：与新生儿红斑狼疮相关的病例报告及文献复习
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