The DOI matches its Crossref record; 5 of the 5 references it cites were found in a registry or an index.
Macrophage Activation Syndrome: different mechanisms leading to a one clinical syndrome
Open accessAbstract
BACKGROUND: Macrophage activation syndrome (MAS) is a severe complication of rheumatic disease in childhood, particularly in systemic Juvenile Idiopathic Arthritis (sJIA). It is characterize by an uncontrolled activation and proliferation of T lymphocytes and macrophages. MAIN CONTENT: MAS is currently classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH). The reason is that MAS shares clinical and laboratory features with primary genetic HLH (pHLH). In this context is conceivable that some of the pathogenic mechanisms of pHLH may be involved in other forms of HLH. Heterozygosity for mutations of genes involved in pHLH may lead to a cytotoxic defect and to a development of clinical overt disease. But other different contributors might be involved to the development of MAS such as infections or underlying inflammation. In MAS, the inflammatory status of the patient is a major contributor of the disease. Indeed, the majority of the MAS episodes occurs during active disease phases or at disease onset. In addition, recent evidence in animals and humans suggest that genetics may also play a major role in contributing to hyperinflammation and particularly to macrophages hyper-responses. CONCLUSIONS: We hypothesize that HLH may be one unique clinical syndrome, to whose generation different mechanisms may contribute, and maintained by one final effector mechanism.
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Repeated TLR9 stimulation results in macrophage activation syndrome–like disease in mice
Behrens, E. M., Canna, S. W., Slade, K., Rao, S., Kreiger, P. A., Paessler, M., Kambayashi, T., & Koretzky, G. A. (2011) · Journal of Clinical Investigation · doi:10.1172/jci43157 · Cited by 392
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McGonagle, D., Sharif, K., O'Regan, A., & Bridgewood, C. (2020) · Autoimmunity Reviews · doi:10.1016/j.autrev.2020.102537 · Cited by 1,654
Jamilloux, Y., Henry, T., Belot, A., Viel, S., Fauter, M., El Jammal, T., Walzer, T., François, B., & Sève, P. (2020) · Autoimmunity Reviews · doi:10.1016/j.autrev.2020.102567 · Cited by 658
Sardu, C., Gambardella, J., Morelli, M. B., Wang, X., Marfella, R., & Santulli, G. (2020) · Journal of Clinical Medicine · doi:10.3390/jcm9051417 · Cited by 592
The Role of Monocytes and Macrophages in Autoimmune Diseases: A Comprehensive Review
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Inhibition of Bruton tyrosine kinase in patients with severe COVID-19
Roschewski, M., Lionakis, M. S., Sharman, J. P., Roswarski, J., Goy, A., Monticelli, M. A., Roshon, M., Wrzesinski, S. H., Desai, J. V., Zarakas, M. A., et al. (2020) · Science Immunology · doi:10.1126/sciimmunol.abd0110 · Cited by 357
Metadata
- Authors
- Bracaglia, C., Prencipe, G., & De Benedetti, F.
- Venue
- Pediatric Rheumatology
- Published
- 2017-01-17
- Volume
- 15
- Issue
- 1
- Pages
- 5-5
- ISSN
- 1546-0096 (ISSN-L)
- OpenAlex
- W2574674925
- Cited by
- 160
- References
- 5
- FWCI
- 13.39
- Type
- journal article
- Language
- en
- Open access
- gold
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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