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Macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis
BACKGROUND: Macrophage activation syndrome (MAS) is a severe, potentially life-threatening condition induced by chronic rheumatic diseases, especially systemic-onset juvenile idiopathic arthritis (SoJIA) in childhood. This study aimed to analyze the clinical and laboratory characteristics of systemi...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer-Verlag
2008
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7102092/ https://www.ncbi.nlm.nih.gov/pubmed/18661762 http://dx.doi.org/10.1007/s12519-008-0018-6 |
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author | Zeng, Hua-Song Xiong, Xiao-Yan Wei, Yan-Dan Wang, Hong-Wei Luo, Xiao-Ping |
author_facet | Zeng, Hua-Song Xiong, Xiao-Yan Wei, Yan-Dan Wang, Hong-Wei Luo, Xiao-Ping |
author_sort | Zeng, Hua-Song |
collection | PubMed |
description | BACKGROUND: Macrophage activation syndrome (MAS) is a severe, potentially life-threatening condition induced by chronic rheumatic diseases, especially systemic-onset juvenile idiopathic arthritis (SoJIA) in childhood. This study aimed to analyze the clinical and laboratory characteristics of systemic-onset juvenile idiopathic arthritis (SoJIA) with macrophage activation syndrome (MAS) in 13 patients. METHODS: Clinical and laboratory data of 13 SoJIA patients with MAS treated in our hospital from January 2003 to October 2007 were analyzed. RESULTS: In the 13 patients, 9 were boys and 4 girls aged from 5 months to 12 years. Clinical manifestations were of no typical characteristics including persistent fever, anemia, arthritis, hepatosplenomegaly, lymph-adenopathy, dysfunction of the liver, abnormal fat metabolism, and hemophagocytic cells in the bone marrow. Two patients experienced acute respiratory distress syndrome, two had mutiorgan failure, and three died. The perforin A91V (NCBI:SNP rs35947132) gene in 6 patients was normal. Glucocorticoid and immunoimpressive therapy were effective in all patients and plasmapheresis used in one severe patient was also effective. CONCLUSIONS: MAS is a serious complication of JIA, especially systemic-onset juvenile idiopathic arthritis. It is essentially important to recognize and treat MAS earlier in order to lower the mortality. |
format | Online Article Text |
id | pubmed-7102092 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2008 |
publisher | Springer-Verlag |
record_format | MEDLINE/PubMed |
spelling | pubmed-71020922020-03-31 Macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis Zeng, Hua-Song Xiong, Xiao-Yan Wei, Yan-Dan Wang, Hong-Wei Luo, Xiao-Ping World J Pediatr Original Article BACKGROUND: Macrophage activation syndrome (MAS) is a severe, potentially life-threatening condition induced by chronic rheumatic diseases, especially systemic-onset juvenile idiopathic arthritis (SoJIA) in childhood. This study aimed to analyze the clinical and laboratory characteristics of systemic-onset juvenile idiopathic arthritis (SoJIA) with macrophage activation syndrome (MAS) in 13 patients. METHODS: Clinical and laboratory data of 13 SoJIA patients with MAS treated in our hospital from January 2003 to October 2007 were analyzed. RESULTS: In the 13 patients, 9 were boys and 4 girls aged from 5 months to 12 years. Clinical manifestations were of no typical characteristics including persistent fever, anemia, arthritis, hepatosplenomegaly, lymph-adenopathy, dysfunction of the liver, abnormal fat metabolism, and hemophagocytic cells in the bone marrow. Two patients experienced acute respiratory distress syndrome, two had mutiorgan failure, and three died. The perforin A91V (NCBI:SNP rs35947132) gene in 6 patients was normal. Glucocorticoid and immunoimpressive therapy were effective in all patients and plasmapheresis used in one severe patient was also effective. CONCLUSIONS: MAS is a serious complication of JIA, especially systemic-onset juvenile idiopathic arthritis. It is essentially important to recognize and treat MAS earlier in order to lower the mortality. Springer-Verlag 2008-07-16 2008 /pmc/articles/PMC7102092/ /pubmed/18661762 http://dx.doi.org/10.1007/s12519-008-0018-6 Text en © Springer 2008 This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic. |
spellingShingle | Original Article Zeng, Hua-Song Xiong, Xiao-Yan Wei, Yan-Dan Wang, Hong-Wei Luo, Xiao-Ping Macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis |
title | Macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis |
title_full | Macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis |
title_fullStr | Macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis |
title_full_unstemmed | Macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis |
title_short | Macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis |
title_sort | macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7102092/ https://www.ncbi.nlm.nih.gov/pubmed/18661762 http://dx.doi.org/10.1007/s12519-008-0018-6 |
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