Macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis (sJIA) is difficult to distinguish from active sJIA or sepsis. A collaborative effort scoring the performance of different clinical diagnostic criteria provides valuable guidance to clinicians, but challenges in the diagnosis of sJIA-related MAS remain.
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References
Davi, S. et al. Performance of current guidelines for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. Arthritis Rheum. http://dx.doi.org/10.1002/art.38769.
Janka, G. E. Familial and acquired hemophagocytic lymphohistiocytosis. Annu. Rev. Med. 63, 233–246 (2012).
Mellins, E. D., Macaubas, C. & Grom, A. A. Pathogenesis of systemic juvenile idiopathic arthritis: some answers, more questions. Nat. Rev. Rheumatol. 7, 416–426 (2011).
de Jager, W. et al. Defective phosphorylation of interleukin-18 receptor β causes impaired natural killer cell function in systemic-onset juvenile idiopathic arthritis. Arthritis Rheum. 60, 2782–2793 (2009).
Henter, J. I. et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr. Blood Cancer 48, 124–131 (2007).
Ravelli, A. et al. Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. J. Pediatr. 146, 598–604 (2005).
Bleesing, J. et al. The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor α-chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis. Arthritis Rheum. 56, 965–971 (2007).
Gorelik, M. et al. Follistatin-like protein 1 and the ferritin/erythrocyte sedimentation rate ratio are potential biomarkers for dysregulated gene expression and macrophage activation syndrome in systemic juvenile idiopathic arthritis. J. Rheumatol. 40, 1191–1199 (2013).
Shimizu, M. et al. Distinct cytokine profiles of systemic-onset juvenile idiopathic arthritis-associated macrophage activation syndrome with particular emphasis on the role of interleukin-18 in its pathogenesis. Rheumatology (Oxford) 49, 1645–1653 (2010).
Frosch, M. et al. The myeloid-related proteins 8 and 14 complex, a novel ligand of Toll-like receptor 4, and interleukin-1β form a positive feedback mechanism in systemic-onset juvenile idiopathic arthritis. Arthritis Rheum. 60, 883–891 (2009).
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The authors acknowledge V. Boom for performing a literature search on biomarkers in macrophage activation syndrome.
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Vastert, S., Prakken, B. Diagnosing macrophage activation syndrome in systemic JIA. Nat Rev Rheumatol 10, 640–642 (2014). https://doi.org/10.1038/nrrheum.2014.143
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DOI: https://doi.org/10.1038/nrrheum.2014.143
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