ArticleBMC medicine2016
Adult-onset Still's disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers.
Article in BMC medicine, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 88 papers, 1 of them a synthesis that pooled it.
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Who cites it
88 citing papers in PubMed, 1 synthesis or guideline pooled it, 174 citations in OpenAlex.
- Mortality risk and causes of death in exogenous Cushing's syndrome: a systematic review and meta-analysis.The Journal of clinical endocrinology and metabolism · 2026Pooled it
- Canakinumab for Treatment of Adult-Onset Still's Disease to Achieve Reduction of Arthritic Manifestation (CONSIDER): phase II, randomised, double-blind, placebo-controlled, multicentre, investigator-initiated trial.Annals of the rheumatic diseases · 2020Trial
- The EULAR points to consider regarding the development of criteria for the assessment of the disease activity in adult-onset Still's disease.EULAR rheumatology open · 2026Article
- Refractory Relapsing-Remitting Adult-Onset Still's Disease in an Adolescent Female: A Rare Case Report.Clinical case reports · 2026Article
- Article
- Review
- Machine learning for predicting macrophage activation syndrome in adult patients with Still's disease.Journal of translational autoimmunity · 2026Article
- Article
- When Fever Defies Diagnosis: Wissler-Fanconi Syndrome as an Atypical Presentation of Adult-Onset Still's Disease.Clinical case reports · 2026Article
- Unfolding dermatological spectrum of Still's disease: a cohort study from the International AIDA Network Still's Disease Registry.Rheumatology (Oxford, England) · 2026Article
- Tocilizumab combined with short-term high-dose glucocorticoids for rapid disease activity control and glucocorticoid reduction in adult-onset Still's disease: a single-center retrospective study.Frontiers in immunology · 2026Article
- Article
- 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
- Real-world therapeutic strategies in active adult-onset Still's disease: clinical insights from the GIRRCS-AOSD study group.Frontiers in immunology · 2026Article
- Advances in the study of low-density neutrophils in rheumatic diseases.Frontiers in immunology · 2026Review
- Advancing Precision Medicine in Adult-Onset Still's Disease: Insights into Biomarkers, Therapies, and COVID-19 Impacts.Mediterranean journal of rheumatology · 2025Review
- Multimodal Noninvasive Assessment of C-Reactive Protein for Systemic Inflammation in Adults: Cross-Sectional Study.JMIR formative research · 2025Article
- Adult-Onset Still's Disease: A Rare Cause of Acute Severe Mitral Regurgitation.Anatolian journal of cardiology · 2025Article
- The Risk Factors of Refractory Adult-Onset Still's Disease.International journal of hepatology · 2025Article
- Prediction of 90 day readmission in heart failure with preserved ejection fraction by interpretable machine learning.ESC heart failure · 2024Article
28 more citing papers are in PubMed but not listed here.
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Authors and funding
14 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAdult-onset Still's disease (AOSD) is rare inflammatory disease of unknown etiology that usually affects young adults. The more common clinical manifestations are spiking fevers, arthritis, evanescent rash, elevated liver enzymes, lymphadenopathy, hepatosplenomegaly, and serositis. The multi-visceral involvement of the disease and the different complications, such as macrophage activation syndrome, may strongly decrease the life expectancy of AOSD patients.
methodsThis study aimed to identify the positive and negative features correlated with the outcome of patients. A retrospective analysis of AOSD patients prospectively admitted to three rheumatologic centers was performed to identify the clinical features present at the time of diagnosis and to predict the possible outcome. Furthermore, we investigated the as yet to be validated prognostic value of the systemic score previously proposed.
resultsOne hundred consecutive AOSD patients were enrolled. The mean systemic score showed that the majority of patients had a multi-organ involvement. Sixteen patients showed different complications, mainly the macrophage activation syndrome. A strong increase of inflammatory markers was observed. All patients received steroids at different dosages, 55 patients in association with immunosuppressive drugs and 32 in association with biologic agents. Sixteen patients died during the follow-up. Regression analysis showed that the higher values of the systemic score and the presence of AOSD-related complications, assessed at the time of diagnosis, were significantly correlated with patient mortality. A prognostic impact of the systemic score of ≥ 7.0 was reported.
conclusionsOur study showed that a higher systemic score and the presence of AOSD-related complications at the time of diagnosis were significantly associated with mortality. Of note, a cut-off at 7.0 of the systemic score showed a strong prognostic impact in identifying patients at risk of AOSD-related death.
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