Journal List > J Rheum Dis > v.21(2) > 1064171

J Rheum Dis. 2014 Apr;21(2):96-100. Korean.
Published online April 30, 2014.
Copyright © 2014 by The Korean College of Rheumatology
A Case of Macrophage Activation Syndrome Developed in Female Adolescent with Systemic Lupus Erythematosus
Seung Woo Keum, Min Jae Kim, E Young Bae, Seung Beom Han, Nack-Gyun Chung, Dae-Chul Jeong, and Jin Han Kang
Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea.

Corresponding to: Dae-Chul Jeong, Department of Pediatrics, Seoul St. Mary's Hospital, 222, Banpo-daero, Seocho-gu, Seoul 137-701, Korea. Email:
Received January 20, 2013; Revised June 20, 2013; Accepted June 20, 2013.

This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.


Macrophage activation syndrome (MAS) is a severe complication in patients with autoimmune disease. We should consider MAS in patients with autoimmune disease, who present with newly developed fever, and MAS needs proper management due to grave outcome. We report a case of MAS in a 15-year-old adolescent girl, who was newly diagnosed with systemic lupus erythematosus 1 month before the diagnosis of MAS. Her MAS was improved by intensive treatment, including etoposide.

Keywords: Macrophage activation syndrome; Systemic lupus erythematosus; Adolescent


Figure 1
Clinical course of an adolescent girl diagnosed with macrophage activation syndrome developed in systemic lupus erythematosus.
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Table 1
Preliminary guidelines for macrophage activation syndrome as a complication of juvenile systemic lupus erythematosus (10)
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Table 2
Diagnostic guidelines for hemophagocytic lymphohistiocytosis (11)
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