Abstract
Macrophage activation syndrome (MAS), life-threatening syndrome of overwhelming inflammation caused by uncontrolled hyperactivation of macrophages, is a rare complication of childhood systemic inflammatory disorders, affecting most often patients with systemic onset juvenile idiopathic arthritis (soJIA). The diagnosis of MAS in soJIA is challenging due to overlapping manifestations of the two diseases. Because MAS is often a fatal condition, prompt diagnosis and immediate therapeutic intervention are important.
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References
Stephan JL, Zeller J, Hubert P, Herbelin C, Dayer JM, Prieur AM. Macrophage activation syndrome and rheumatic disease in childhood: A report of four new cases. Clin Exp Rheumatol 1993; 11: 451–6.
Ravelli AP, A. Malattia, C. Sala, I, Martini, A. Macrophage activation syndrome in childhood rheumatic diseases. Curr Rheumatol Rev 2007; 2: 225–30.
Villanueva J, Lee S, Giannini EH, Graham TB, Passo MH, Filipovich A, Grom AA. Natural killer cell dysfunction is a distinguishing feature of systemic onset juvenile rheumatoid arthritis and macrophage activation syndrome. Arthritis Res Ther 2005; 7: R30–7.
Avcin T, Tse SM, Schneider R, Ngan B, Silverman ED. Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood. J Pediatr 2006; 148: 683–6.
Athreya BH. Is macrophage activation syndrome a new entity? Clin Exp Rheumatol 2002; 20: 121–3.
Sawhney S, Woo P, Murray KJ. Macrophage activation syndrome: A potentially fatal complication of rheumatic disorders. Arch Dis Child 2001; 85: 421–6.
Behrens EM, Beukelman T, Paessler M, Cron RQ. Occult macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis. J Rheumatol 2007; 34: 1133–8.
Bleesing J, Prada A, Siegel DM, Villanueva J, Olson J, Ilowite NT, Brunner HI, Griffin T, Graham TB, Sherry DD, Passo MH, Ramanan AV, Filipovich A, Grom AA. The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor alpha-chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis. Arthritis Rheum 2007; 56: 965–71.
Stephan JL, Kone-Paut I, Galambrun C, Mouy R, Bader-Meunier B, Prieur AM. Reactive haemophagocytic syndrome in children with inflammatory disorders. A retrospective study of 24 patients. Rheumatology (Oxford) 2001; 40: 1285–92.
Grom AA, Villanueva J, Lee S, Goldmuntz EA, Passo MH, Filipovich A. Natural killer cell dysfunction in patients with systemic-onset juvenile rheumatoid arthritis and macrophage activation syndrome. J Pediatr 2003; 142: 292–6.
Henter JI. Biology and treatment of familial hemophagocytic lymphohistiocytosis: Importance of perforin in lymphocyte-mediated cytotoxicity and triggering of apoptosis. Med Pediatr Oncol 2002; 38: 305–9.
Ramanan AV, Grom AA. Does systemic-onset juvenile idiopathic arthritis belong under juvenile idiopathic arthritis? Rheumatology (Oxford) 2005; 44: 1350–3.
Ravelli A, Magni-Manzoni S, Pistorio A, Besana C, Foti T, Ruperto N, Viola S, Martini A. Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. J Pediatr 2005; 146: 598–604.
Ramanan AV, Schneider R. Macrophage activation syndrome – what's in a name! J Rheumatol 2003; 30: 2513–6.
Henter JI, Elinder G, Ost A. Diagnostic guidelines for hemophagocytic lymphohistiocytosis. The FHL Study Group of the Histiocyte Society. Semin Oncol 1991; 18: 29–33.
Imashuku S, Hlbi S, Todo S. Hemophagocytic lymphohistiocytosis in infancy and childhood. J Pediatr 1997; 130: 352–7.
Janka GE, Schneider EM. Modern management of children with haemophagocytic lymphohistiocytosis. Br J Haematol 2004; 124: 4–14.
Kounami S, Yoshiyama M, Nakayama K, Okuda M, Okuda S, Aoyagi N, Yoshikawa N. Macrophage activation syndrome in children with systemic-onset juvenile chronic arthritis. Acta Haematol 2005; 113: 124–9.
Schaer DJ, Schleiffenbaum B, Kurrer M, Imhof A, Bachli E, Fehr J, Moller HJ, Moestrup SK, Schaffner A. Soluble hemoglobin-haptoglobin scavenger receptor CD163 as a lineage-specific marker in the reactive hemophagocytic syndrome. Eur J Haematol 2005; 74: 6–10.
Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, Ladisch S, McClain K, Webb D, Winiarski J, Janka G. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48: 124–31.
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© 2008 Humana Press, a part of Springer Science+Business Media, LLC
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Berkun, Y., Padeh, S. (2008). Macrophage Activation Syndrome in Juvenile Idiopathic Arthritis. In: Shoenfeld, Y., Cervera, R., Gershwin, M.E. (eds) Diagnostic Criteria in Autoimmune Diseases. Humana Press. https://doi.org/10.1007/978-1-60327-285-8_4
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DOI: https://doi.org/10.1007/978-1-60327-285-8_4
Publisher Name: Humana Press
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