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6/2013 vol. 51
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REVIEW PAPER
Macrophage activation syndrome
Bożena Rojek-Margas
,
 
Beata Śliwowska
,
 
Jolanta Bucka
 
 
 
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Submission date: 2013-07-06
 
 
Final revision date: 2013-09-02
 
 
Acceptance date: 2013-11-18
 
 
Online publication date: 2013-12-24
 
 
Publication date: 2013-12-20
 
 
Reumatologia 2013;51(6):459-466
DOI: https://doi.org/10.5114/reum.2013.39666
 
 
Article (PDF)
References (28)
 
KEYWORDS
macrophage activation syndrome
hemophagocytic syndrome
hemophagocytic lymphohistiocytosis
 
TOPICS
Cytokines, Inflammatory mediators and adaptive immunity in rheumatic diseases
Rheumatoid arthritis- aetiology, pathogenesis, clinical features and treatment
Infection related rheumatoid diseases
 
ABSTRACT
Macrophage activation syndrome (MAS) is a severe complication of systemic connective tissue diseases, particularly systemic juvenile idiopathic arthritis, systemic lupus erythematosus and adult-onset Still’s disease. It is a life-threatening disorder arising from immunoregulation inherited or acquired dysfunction of the NK cells and cytotoxic T-lymphocytes. It is characterized by multi-organ failure with a number of clinical symptoms. So far, there is no standard procedure for treatment. Glucocorticoids, intravenous immunoglobulin, sometimes cyclosporine A and etoposide are used.
 
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Copyright: © Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. This is an Open Access journal, all articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (https://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
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