SynthesisAnnals of the rheumatic diseases2024
Efficacy and safety of therapies for Still's disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR/PReS guidelines for the 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 23 papers, 2 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
23 citing papers in PubMed, 2 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
- Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project.Rheumatology (Oxford, England) · 2025Pooled it
- Two- and 3-year outcomes of the childhood arthritis and rheumatology research alliance FiRst Line Options for sJIA Treatment (FROST) trial.Pediatric rheumatology online journal · 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
- Switching from Originator Tocilizumab to Biosimilar in Giant Cell Arteritis: Evaluation of Effectiveness and Safety in a Multicenter Cohort of 38 Patients.Drugs & aging · 2026Observational
- Pharmacologic management of renal involvement in monogenic autoinflammatory diseases.European journal of medical research · 2026Review
- Still's Disease and Autoinflammation: Positioning an Inflammatory Syndrome on the Autoinflammation-Autoimmunity Spectrum.Current rheumatology reports · 2026Review
- The "cytokine storm" in infection and sepsis: win the battle but lose the war.Military Medical Research · 2026Review
- 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
- 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
- Impact of Interleukin-1 Blockade on the Development of Macrophage Activation Syndrome in Still Disease: Incidence and Diagnostic Validity of the EULAR/ACR/PRINTO 2016 MAS Classification Criteria.Arthritis & rheumatology (Hoboken, N.J.) · 2025Article
- Emapalumab as a Life-Saving Therapy for Macrophage Activation Syndrome in Refractory Systemic Juvenile Idiopathic Arthritis with Trisomy 21.Turkish archives of pediatrics · 2025Article
- Early IL-1 Inhibition in Still's Disease: A Window of Opportunity for Improving Outcomes.ACR open rheumatology · 2025Article
- Article
- Neutrophilic Urticarial Dermatosis: A Window into Systemic Inflammation and Autoimmune Disorders.Clinical reviews in allergy & immunology · 2025Review
- Salvage treatment of AOSD-associated macrophage activation syndrome with bariticinib following conventional immunosuppressive therapy.Clinical rheumatology · 2025Article
- The Golden Card of Interleukin-1 Blockers in Systemic Inflammasomopathies of Childhood.International journal of molecular sciences · 2025Review
- Patients with Adult-Onset Still's Disease in Germany: A Retrospective Analysis of Clinical Characteristics and Treatment Practices Ahead of the Release of the German Recommendations.Journal of clinical medicine · 2025Article
- Macrophage activation syndrome successfully treated with eculizumab and emapalumab: a case report.Frontiers in immunology · 2025Article
- Low-dose emapalumab treatment in refractory macrophage activation syndrome secondary to adult onset still's disease/systemic lupus erythematosus: insights from nine cases.Frontiers in immunology · 2025Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
6 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesTo analyse the efficacy and safety of treatments for Still's disease and macrophage activation syndrome (MAS).
methodsMedline, Embase and Cochrane Library were searched for clinical trials (randomised, randomised controlled trial (RCT), controlled and clinical controlled trial (CCT)), observational studies (retrospective, longitudinal observational retrospective (LOR), prospective and longitudinal observational prospective (LOP)) and systematic reviews (SRs), in which the populations studied were patients with Still's disease and MAS. The intervention was any pharmacological treatment (approved or under evaluation) versus any comparator drug or placebo, and as outcomes, any relevant efficacy and safety event. The risk of bias (RoB) was assessed with the Cochrane RoB and AMSTAR-2 (Assessing the Methodological Quality of Systematic Reviews-2, version 2) for SRs.
results128 full texts were included: 25 RCTs, 1 CCT, 11 SRs published after 2013 and 91 LOP/LOR studies. In Still's disease, interleukin (IL)-1 inhibitors (IL-1i) and IL-6R inhibitors (IL-6i) were the most studied drugs. Two meta-analyses on RCTs showed an OR, to achieve an ARC50 response rate, of 6.02 (95% CI 2.24 to 21.36) and 8.08 (95% CI 1.89 to 34.57) for IL-1i and IL-6Ri, respectively. Retrospective studies showed that early initiation of IL-1i or IL-6i was associated with high rates of clinically inactive disease. In MAS, GCs were employed in all patients, often associated with ciclosporin and/or anakinra. Rates of complete response were reported, with a range from 53% to 100%. Emapalumab was the only drug tested in a CCT, with a complete response of 93%.
conclusionIL-1i and IL-6Ri show the highest level of efficacy in the treatment of Still's disease. For MAS, IL-1 and interferon-γ inhibition appear to be effective on a background of high-dose glucocorticoids.
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