The DOI matches its Crossref record; 10 of the 10 references it cites were found in a registry or an index.
Pathogenesis of systemic juvenile idiopathic arthritis: some answers, more questions
Open accessAbstract
Systemic juvenile idiopathic arthritis (sJIA) has long been recognized as unique among childhood arthritides, because of its distinctive clinical and epidemiological features, including an association with macrophage activation syndrome. Here, we summarize research into sJIA pathogenesis. The triggers of disease are unknown, although infections are suspects. Once initiated, sJIA seems to be driven by innate proinflammatory cytokines. Endogenous Toll-like receptor ligands, including S100 proteins, probably synergize with cytokines to perpetuate inflammation. These and other findings support the hypothesis that sJIA is an autoinflammatory condition. Indeed, IL-1 is implicated as a pivotal cytokine, but the source of excess IL-1 activity remains obscure and the role of IL-1 in chronic arthritis is less clear. Another hypothesis is that a form of hemophagocytic lymphohistiocytosis underlies sJIA, with varying degrees of its expression across the spectrum of disease. Alternatively, sJIA with MAS might be a genetically distinct subtype. Yet another hypothesis proposes that inadequate downregulation of immune activation is central to sJIA, supporting evidence for which includes 'alternative activation' of monocyte and macrophages and possible deficiencies in IL-10 and T regulatory cells. Some altered immune phenotypes persist during clinically inactive disease, which suggests that this stage might represent compensated inflammation. Despite much progress being made, many questions remain, providing fertile ground for future research.
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Cited by
Kaufman, K. M., Linghu, B., Szustakowski, J. D., Husami, A., Yang, F., Zhang, K., Filipovich, A. H., Fall, N., Harley, J. B., Nirmala, N. R., et al. (2014) · Arthritis & Rheumatology · doi:10.1002/art.38793 · Cited by 207
Self-citationAnaemia in inflammatory rheumatic diseases
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The genetics of macrophage activation syndrome
Schulert, G. S., & Cron, R. Q. (2020) · Genes & Immunity · doi:10.1038/s41435-020-0098-4 · Cited by 91
Avau, A., Mitera, T., Put, S., Put, K., Brisse, E., Filtjens, J., Uyttenhove, C., Van Snick, J., Liston, A., Leclercq, G., et al. (2014) · Arthritis & Rheumatology · doi:10.1002/art.38359 · Cited by 90
Pardeo, M., Bracaglia, C., & De Benedetti, F. (2017) · Best Practice & Research Clinical Rheumatology · doi:10.1016/j.berh.2018.02.002 · Cited by 60
Metadata
- Authors
- Mellins, E. D., Macaubas, C., & Grom, A. A.
- Venue
- Nature Reviews Rheumatology
- Published
- 2011-06-07
- Volume
- 7
- Issue
- 7
- Pages
- 416-426
- ISSN
- 1759-4790 (ISSN-L)
- 1759-4804
- OpenAlex
- W2010387075
- Cited by
- 330
- References
- 10
- FWCI
- 2.11
- Type
- journal article
- Language
- en
- Open access
- green
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