Adult-onset Still disease
Quotidian fever, evanescent salmon-pink rash, arthralgia and a neutrophilia with a very high ferritin. A diagnosis of exclusion (Yamaguchi criteria), treated early with IL-1 or IL-6 blockade under the 2024 EULAR/PReS treat-to-target plan, with macrophage activation syndrome as the complication that kills.
Tier 2Condition
Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made
At a glance
- Adult-onset Still disease (AOSD) is a systemic autoinflammatory disease driven by IL-1, IL-6 and IL-18. EULAR/PReS 2024 treats it and systemic JIA as one disease, Still disease.
- Bimodal onset at 15 to 25 and 36 to 46 years. It presents as pyrexia of unknown origin with sore throat, rash and joint pain.
- No diagnostic test. It is a diagnosis of exclusion; the Yamaguchi criteria are the most sensitive.
Loading the rest of the note…
Finished reading?
Sources
- Therapeutic Guidelines: Rheumatology (adult-onset Still disease)AU/NZTherapeutic Guidelines Limited (eTG complete), current online version, accessed November 2026
- Fautrel B et al. EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease. Ann Rheum Dis 2024;83:1614-27EULAR and PReS, 2024 (current as of November 2026)
- De Matteis A et al. Systemic juvenile idiopathic arthritis and adult-onset Still's disease are the same disease: evidence from systematic reviews and meta-analyses informing the 2023 EULAR/PReS recommendations. Ann Rheum Dis 2024Annals of the Rheumatic Diseases, 2024
- Yamaguchi M et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol 1992;19:424-30Journal of Rheumatology, 1992
- Fautrel B et al. Proposal for a new set of classification criteria for adult-onset Still disease. Medicine (Baltimore) 2002;81:194-200Medicine (Baltimore), 2002
- Last updated
- 14 November 2026
What changed
- 14 November 2026 Turnstile bot check on sign-in and signed-out feedback