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一例以巨噬细胞活化综合征为系统性红斑狼疮首发表现的病例。

A Case of Macrophage Activation Syndrome Manifesting as the Initial Presentation of Systemic Lupus Erythematosus.

作者信息

Suwal Amar, Shrestha Biraj, Paudel Anish, Paudel Rubina, Basnet Sijan

机构信息

Internal medicine, Reading Hospital-Tower Health, Reading, USA.

Internal Medicine, Reading Hospital-Tower Health, Reading, USA.

出版信息

Cureus. 2022 Jun 27;14(6):e26375. doi: 10.7759/cureus.26375. eCollection 2022 Jun.

DOI:10.7759/cureus.26375
PMID:35911271
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9328934/
Abstract

Macrophage activation syndrome (MAS) is a potentially fatal complication of an autoimmune rheumatologic disease characterized by overwhelming inflammation, multiorgan failure, and high mortality if untreated. We report a rare case of a 56-year-old man who presented with fever for three weeks and had a constellation of clinical features and laboratory findings, meeting the diagnostic criteria for systemic lupus erythematosus (SLE) and SLE-associated MAS. He was treated with high dose intravenous corticosteroid and hydroxychloroquine, resulting in resolution of fever and dramatic clinical improvement.

摘要

巨噬细胞活化综合征(MAS)是自身免疫性风湿性疾病的一种潜在致命并发症,其特征为炎症反应剧烈、多器官功能衰竭,若不治疗死亡率很高。我们报告一例罕见病例,一名56岁男性,发热三周,具有一系列临床特征和实验室检查结果,符合系统性红斑狼疮(SLE)及SLE相关MAS的诊断标准。他接受了大剂量静脉注射皮质类固醇和羟氯喹治疗,发热消退,临床症状显著改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32c6/9328934/408574b4f396/cureus-0014-00000026375-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32c6/9328934/408574b4f396/cureus-0014-00000026375-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32c6/9328934/408574b4f396/cureus-0014-00000026375-i01.jpg

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本文引用的文献

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Cureus. 2021 Oct 16;13(10):e18822. doi: 10.7759/cureus.18822. eCollection 2021 Oct.
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Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: A study of 103 episodes in 89 adult patients.系统性红斑狼疮相关巨噬细胞活化综合征的临床特征和治疗管理:89 例成人患者 103 个发作病例研究。
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系统性红斑狼疮中巨噬细胞活化综合征:中国多中心病例对照研究。
Clin Rheumatol. 2018 Jan;37(1):93-100. doi: 10.1007/s10067-017-3625-6. Epub 2017 Apr 13.
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In-hospital mortality in febrile lupus patients based on 2016 EULAR/ACR/PRINTO classification criteria for macrophage activation syndrome.基于 2016 年 EULAR/ACR/PRINTO 分类标准的巨噬细胞活化综合征的发热性狼疮患者住院死亡率。
Semin Arthritis Rheum. 2017 Oct;47(2):216-221. doi: 10.1016/j.semarthrit.2017.02.002. Epub 2017 Feb 8.
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Unusual Association of Hemophagocytic Lymphohistiocytosis in Systemic Lupus Erythematosus: Cases Reported at Tertiary Care Center.系统性红斑狼疮合并噬血细胞性淋巴组织细胞增生症的罕见关联:三级医疗中心报告的病例
Am J Case Rep. 2016 Oct 13;17:739-744. doi: 10.12659/ajcr.899433.
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How I treat hemophagocytic lymphohistiocytosis in the adult patient.成人噬血细胞性淋巴组织细胞增生症的治疗方法。
Blood. 2015 May 7;125(19):2908-14. doi: 10.1182/blood-2015-01-551622. Epub 2015 Mar 10.
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