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Adult-onset still disease is a systemic illness primarily affecting young adults. Clinical presentation includes high spiking fevers, arthralgia, sore throat, and evanescent salmon-pink patches favoring sites of pressure that correlates with fever spikes. Classic treatment typically involves systemic corticosteroids, but newer targeted biologic agents hold promise. In addition to the therapies listed below, Rituximab and the IL-18 inhibitor Tadekinig alfa have been reported, but require more data. See Table 52-1.
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Levels of evidence are based on the Journal of the American Academy of Dermatology guidelines: level IA evidence includes evidence from meta-analysis of randomized controlled trials; level IB evidence includes evidence from ≥1 randomized controlled trial; level IIA evidence includes evidence from ≥1 controlled study without randomization; level IIB evidence includes evidence from ≥1 other type of experimental study; level III evidence includes evidence from nonexperimental descriptive studies, such as comparative studies, correlation studies, and case control studies; and level IV evidence includes evidence from expert committee reports or opinions or clinical experience of respected authorities, or both.
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Castañeda
S, Blanco
R, González-Gay
MA. Adult-onset Still’s disease: advances in the treatment. Best Pract Res Clin Rheumatol. 2016;30(2):222–238. doi: 10.1016/j.berh.2016.08.003.