GuidelineAnnals of the rheumatic diseases2024
EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease.
Guideline in Annals of the rheumatic diseases, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 118 papers, 5 of them syntheses that pooled it.
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Who cites it
118 citing papers in PubMed, 5 syntheses or guidelines pooled it.
- The Asia-Pacific League of Associations for Rheumatology Consensus Recommendations on the Management of Systemic Juvenile Idiopathic Arthritis (Juvenile Still's Disease).International journal of rheumatic diseases · 2026Guideline
- Australian clinical practice guideline: diagnosis and treatment of idiopathic multicentric Castleman disease.Internal medicine journal · 2026Guideline
- Early intensification versus step-up biologic strategies in systemic juvenile idiopathic arthritis: a Bayesian network meta-analysis of remission and safety outcomes.Pediatric rheumatology online journal · 2026Pooled it
- Clinical Features and Outcome Measures Across Still Disease (Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still Disease) Cohorts Worldwide: A Systematic Literature Review.The Journal of rheumatology · 2026Pooled it
- Long-Term Safety of Anti-Interleukin-1 Medications in Children with Rheumatic Diseases: a Systematic Review.Paediatric drugs · 2025Pooled it
- Serum cytokine levels towards precision medicine in Still's disease: a subanalysis of a randomized controlled trial of tocilizumab.Rheumatology (Oxford, England) · 2025Trial
- Skin biopsy in adult-onset Still's disease: A specific, rapid, and widely available diagnostic test.Journal of human immunity · 2026Article
- Identification of genetic variants associated with monogenic autoinflammatory diseases in patients with Still's disease using next-generation sequencing.Immunologic research · 2026Article
- A Rare RIPK3 Variant Enhances Necroptosis and Promotes Inflammation in a Still Disease-Like Autoinflammatory Syndrome.Arthritis & rheumatology (Hoboken, N.J.) · 2026Article
- Advancements in Diagnosis and Care for Immune Dysregulation and Inborn Errors of Immunity in Pediatric Rheumatology: Our 50-Year Journey.Arthritis care & research · 2026Review
- Adult-onset Still's disease in an 83-year-old patient with positive antinuclear antibodies: a case report.Zeitschrift fur Rheumatologie · 2026Article
- Comparative evaluation of disease activity measures for adult-onset Still's disease: a multicentre, retrospective, cohort study.RMD open · 2026Article
- A Multifaceted Interplay Among Hemophagocytosis, Interleukin-18, and Type I Interferon Distinguishes Still Disease From Other Autoinflammatory Diseases.Arthritis & rheumatology (Hoboken, N.J.) · 2026Article
- Article
- Diagnostic performance of HScore for macrophage activation syndrome complicating Still's disease in adults: a multicentre case-control study.Rheumatology (Oxford, England) · 2026Article
- Macrophage Activation Syndrome Associated with Adult-onset Still's Disease Successfully Treated with Plasma Exchange and Canakinumab.Internal medicine (Tokyo, Japan) · 2026Article
- Early Differential Diagnosis of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome in Children: A Clinical Prediction Model Based on 106 Patients.Pathogens (Basel, Switzerland) · 2026Article
- Type I Interferon Signature is Associated With Lung Disease, Drug-Associated Immune Reactions, and Genetic Variation in Interferon-Linked Pathways in Still Disease.Arthritis & rheumatology (Hoboken, N.J.) · 2026Article
- Comparison of Systemic Corticosteroid Therapy Practices Between Pediatric and Adult Rheumatologists: A Multicenter Survey Study.Rheumatology and therapy · 2026Article
- Serum ferritin as a predictor of major adverse cardiovascular events in Takayasu arteritis: a two-center retrospective cohort study.Clinical rheumatology · 2026Observational
58 more citing papers are in PubMed but not listed here.
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Authors and funding
33 authors.
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Abstract
Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are considered the same disease, but a common approach for diagnosis and management is still missing.
methodsIn May 2022, EULAR and PReS endorsed a proposal for a joint task force (TF) to develop recommendations for the diagnosis and management of sJIA and AOSD. The TF agreed during a first meeting to address four topics: similarity between sJIA and AOSD, diagnostic biomarkers, therapeutic targets and strategies and complications including macrophage activation syndrome (MAS). Systematic literature reviews were conducted accordingly.
resultsThe TF based their recommendations on four overarching principles, highlighting notably that sJIA and AOSD are one disease, to be designated by one name, Still's disease.Fourteen specific recommendations were issued. Two therapeutic targets were defined: clinically inactive disease (CID) and remission, that is, CID maintained for at least 6 months. The optimal therapeutic strategy relies on early use of interleukin (IL-1 or IL-6 inhibitors associated to short duration glucocorticoid (GC). MAS treatment should rely on high-dose GCs, IL-1 inhibitors, ciclosporin and interferon-γ inhibitors. A specific concern rose recently with cases of severe lung disease in children with Still's disease, for which T cell directed immunosuppressant are suggested. The recommendations emphasised the key role of expert centres for difficult-to-treat patients. All overarching principles and recommendations were agreed by over 80% of the TF experts with a high level of agreement.
conclusionThese recommendations are the first consensus for the diagnosis and management of children and adults with Still's disease.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.