The DOI matches its Crossref record; 85 of the 85 references it cites were found in a registry or an index.
Adult-onset Still's disease
Open accessAbstract
First described in 1971, adult-onset Still's disease (AOSD) is a rare multisystemic disorder considered as a complex (multigenic) autoinflammatory syndrome. A genetic background would confer susceptibility to the development of autoinflammatory reactions to environmental triggers. Macrophage and neutrophil activation is a hallmark of AOSD which can lead to a reactive hemophagocytic lymphohistiocytosis. As in the latter disease, the cytotoxic function of natural killer cells is decreased in patients with active AOSD. IL-18 and IL-1β, two proinflammatory cytokines processed through the inflammasome machinery, are key factors in the pathogenesis of AOSD; they cause IL-6 and Th1 cytokine secretion as well as NK cell dysregulation leading to macrophage activation. The clinico-biological picture of AOSD usually includes high spiking fever with joint symptoms, evanescent skin rash, sore throat, striking neutrophilic leukocytosis, hyperferritinemia with collapsed glycosylated ferritin (<20%), and abnormal liver function tests. According to the clinical presentation of the disease at diagnosis, two AOSD phenotypes may be distinguished: i) a highly symptomatic, systemic and feverish one, which would evolve into a systemic (mono- or polycyclic) pattern; ii) a more indolent one with arthritis in the foreground and poor systemic symptomatology, which would evolve into a chronic articular pattern. Steroid- and methotrexate-refractory AOSD cases benefit now from recent insights into autoinflammatory disorders: anakinra seems to be an efficient, well tolerated, steroid-sparing treatment in systemic patterns; tocilizumab seems efficient in AOSD with active arthritis and systemic symptoms while TNFα-blockers could be interesting in chronic polyarticular refractory AOSD.
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Cites
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Cited by
Overview of the IL ‐1 family in innate inflammation and acquired immunity
Dinarello, C. A. (2018) · Immunological Reviews · doi:10.1111/imr.12621 · Cited by 1,866
Mechanisms, biomarkers and targets for adult-onset Still’s disease
Feist, E., Mitrovic, S., & Fautrel, B. (2018) · Nature Reviews Rheumatology · doi:10.1038/s41584-018-0081-x · Cited by 394
JAK inhibitors for the treatment of autoimmune and inflammatory diseases
Jamilloux, Y., El Jammal, T., Vuitton, L., Gerfaud-Valentin, M., Kerever, S., & Sève, P. (2019) · Autoimmunity Reviews · doi:10.1016/j.autrev.2019.102390 · Cited by 264
Self-citationAdult-onset Still’s disease biological treatment strategy may depend on the phenotypic dichotomy
Vercruysse, F., Barnetche, T., Lazaro, E., Shipley, E., Lifermann, F., Balageas, A., Delbrel, X., Fautrel, B., Richez, C., Schaeverbeke, T., et al. (2019) · Arthritis Research & Therapy · doi:10.1186/s13075-019-1838-6 · Cited by 74
Metadata
- Authors
- Gerfaud-Valentin, M., Jamilloux, Y., Iwaz, J., & Sève, P.
- Venue
- Autoimmunity Reviews
- Publisher
- Elsevier BV
- Published
- 2014-07
- Volume
- 13
- Issue
- 7
- Pages
- 708-722
- ISSN
- 1568-9972 (ISSN-L)
- OpenAlex
- W2913532246
- Cited by
- 518
- References
- 85
- FWCI
- 20.20
- Type
- journal article
- Language
- en
- Open access
- hybrid
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