Macrophage activation syndrome as a presenting feature in juvenile systemic lupus erythematosus.

IF 1.9 4区 医学 Q3 RHEUMATOLOGY
Lupus Pub Date : 2024-10-01 Epub Date: 2024-08-07 DOI:10.1177/09612033241272972
Ishwarya Ramadoss, Pirahalathan Rengabashyam, Mythili Seetharaman Varadhan, Arul R Ponniah Subramanian
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引用次数: 0

Abstract

Background: Macrophage activation syndrome (MAS) is an acquired form of hemo phagocytic lymphohistiocytosis (HLH) and is usually associated with infections, autoimmune, auto inflammatory syndromes and malignancies.

Case details: A 14 year old girl presented with sub-acute onset of fever with lymphadenopathy, pancytopenia,high ferritin values and a falling erythrocyte sedimentation rate. She was evaluated with relevant laboratory tests that was suggestive of systemic Lupus erythematosus and associated macrophage activation syndrome She recovered with immunosuppressive therapy and other supportive care.

Conclusion: There is a need for a high index of suspicion of occult MAS and MAS in patients with systemic lupus erythematosus as it may be an initial presentation. Delay in diagnosis and initiation of treatment can lead to a higher mortality.

作为幼年系统性红斑狼疮一种表现特征的巨噬细胞活化综合征。
背景:巨噬细胞活化综合征(MAS)是一种获得性血吞噬细胞淋巴组织细胞增多症(HLH),通常与感染、自身免疫、自身炎症综合征和恶性肿瘤有关:一名 14 岁女孩因亚急性发热伴淋巴结肿大、泛发性、高铁蛋白值和红细胞沉降率下降而就诊。对她进行了相关的实验室检查,结果提示她患有系统性红斑狼疮和相关的巨噬细胞活化综合征:需要高度怀疑系统性红斑狼疮患者的隐匿性巨噬细胞活化综合征和巨噬细胞活化综合征,因为这可能是患者的最初表现。延误诊断和治疗会导致更高的死亡率。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Lupus
Lupus 医学-风湿病学
CiteScore
4.20
自引率
11.50%
发文量
225
审稿时长
1 months
期刊介绍: The only fully peer reviewed international journal devoted exclusively to lupus (and related disease) research. Lupus includes the most promising new clinical and laboratory-based studies from leading specialists in all lupus-related disciplines. Invaluable reading, with extended coverage, lupus-related disciplines include: Rheumatology, Dermatology, Immunology, Obstetrics, Psychiatry and Cardiovascular Research…
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