SynthesisAnnals of the rheumatic diseases2024
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 for the diagnosis and management of Still's disease.
Synthesis 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 29 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
29 citing papers in PubMed, 1 synthesis or guideline pooled it.
- EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease.Annals of the rheumatic diseases · 2024Guideline
- Construction and evaluation of a chronic arthritis animal model induced by persistent activation of toll-like receptors via lipopolysaccharide stimulation.Animal models and experimental medicine · 2026Article
- Comparative evaluation of disease activity measures for adult-onset Still's disease: a multicentre, retrospective, cohort study.RMD open · 2026Article
- Diagnostic performance of HScore for macrophage activation syndrome complicating Still's disease in adults: a multicentre case-control study.Rheumatology (Oxford, England) · 2026Article
- Article
- The value of C-X-C motif chemokine ligand 9 (CXCL9) in the differential diagnosis and management of haemophagocytic lymphohistiocytosis.British journal of haematology · 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
- Adjunctive Therapeutic Plasma Exchange in Refractory Adult-Onset Still's Disease Complicated by Secondary Macrophage Activation Syndrome: A Single-Center Experience.Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy · 2026Article
- Hepatic Manifestations in Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome.The Journal of rheumatology · 2026Article
- Pathogenic and genetic landscape of Still's disease across ages, with new insights into age-related IL-18 patterns.Arthritis research & therapy · 2026Review
- The gut-joint axis in juvenile idiopathic arthritis.Arthritis research & therapy · 2026Review
- Efficacy of combined JAK1/2 inhibition and cyclosporine in paediatric-onset Still's disease with lung involvement: a case report.Pediatric rheumatology online journal · 2026Article
- Reliability and Construct Validity of the Physician's Global Assessment of Lung Disease in Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease.ACR open rheumatology · 2026Article
- Association of elevated serum active IL-18 levels with cytokine profiles and clinical features in adult-onset still's disease.Frontiers in immunology · 2026Article
- Adult-Onset Still's Disease in a Patient with Macrophage Activation Syndrome and Pre-Disseminated Intravascular Coagulation: A Case Report and Literature Review.Journal of inflammation research · 2026Article
- Application of machine learning techniques to explore the occurrence of macrophage activation syndrome in Still's disease: results from the GIRRCS AOSD Study Group and the AIDA Network Still's Disease Registry.Frontiers in immunology · 2026Observational
- Diagnostic pitfalls in systemic juvenile idiopathic arthritis: insights from 6 misdiagnosed cases.Frontiers in pediatrics · 2026Article
- Article
- Distinct immune activation patterns in adult-onset Still's disease with fungal infections.Frontiers in immunology · 2026Article
- Holistic management of juvenile idiopathic arthritis across all ages: British Society for Rheumatology Guideline scope.Rheumatology advances in practice · 2026Review
Corrections and comments
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Authors and funding
6 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesTo analyse the similarity in clinical manifestations and laboratory findings between systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD).
methodsThree systematic reviews (SR) were performed. One included cohort studies comparing sJIA versus AOSD that described clinical and biological manifestations with at least 20 patients in each group (SR1). The second identified studies of biomarkers in both diseases and their diagnostic performance (SR2). The last focused on diagnostic biomarkers for macrophage activation syndrome (MAS, SR3). Medline (PubMed), Embase and Cochrane Library were systematically searched. The risk of bias was assessed with an adapted form of the Hoy scale for prevalence studies in SR1 and the Quality Assessment of Diagnostic Accuracy Studies-2 in SR2 and SR3. We performed meta-analyses of proportions for the qualitative descriptors.
resultsEight studies were included in SR1 (n=1010 participants), 33 in SR2 and 10 in SR3. The pooled prevalence of clinical manifestations did not differ between sJIA and AOSD, except for myalgia, sore throat and weight loss, which were more frequent in AOSD than sJIA because they are likely ascertained incompletely in sJIA, especially in young children. Except for AA amyloidosis, more frequent in sJIA than AOSD, the prevalence of complications did not differ, nor did the prevalence of biological findings. Ferritin, S100 proteins and interleukin-18 (IL-18) were the most frequently used diagnostic biomarkers, with similar diagnostic performance. For MAS diagnosis, novel biomarkers such as IL-18, C-X-C motif ligand 9, adenosine deaminase 2 activity and activated T cells seemed promising.
conclusionOur results argue for a continuum between sJIA and AOSD. PROSPERO REGISTRATION NUMBER: CRD42022374240 and CRD42024534021.
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