SynthesisRheumatology (Oxford, England)2025
Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project.
Synthesis in Rheumatology (Oxford, England), 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
What it found
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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.
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Who cites it
24 citing papers in PubMed.
- Interferon-γ as a predictive biomarker for macrophage activation syndrome in systemic JIA: a chinese multicenter study.Clinical rheumatology · 2026Article
- Hemophagocytic lymphohistiocytosis in critically ill patients: A guide for clinicians.World journal of critical care medicine · 2026Review
- Macrophage activation syndrome in systemic lupus erythematous and Still's disease: distinct phenotypes shaped by the underlying diseases.Rheumatology (Oxford, England) · 2026Article
- Breaking the storm: canakinumab as salvage therapy for refractory macrophage activation syndrome in pediatric systemic lupus erythematosus-a case report.Pediatric rheumatology online journal · 2026Article
- Macrophage activation syndrome in systemic lupus erythematosus: risk factors and outcomes.Clinical rheumatology · 2026Article
- Novel Organelle-Based Intracellular Immunity with Mechanistic and Therapeutic Implications.Barrier immunity · 2026Article
- 2016 sJIA-MAS criteria and a step-up therapeutic approach in KD-MAS: insights from a combined cohort and literature review.RMD open · 2026Review
- When Rarity Hits Twice: Hemophagocytic Lymphohistiocytosis in Kabuki Syndrome-A Case Report From Palestine.Clinical case reports · 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
- Consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis in the Gulf Cooperation Council: a modified Delphi approach.Frontiers in immunology · 2026Article
- Systemic Lupus Erythematosus with Nephritis: a Macrophage Activation Syndrome Heralding a New Disease Difficult to Diagnose.Mediterranean journal of hematology and infectious diseases · 2026Article
- Case Report: Ruxolitinib for systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome: two pediatric cases and literature review.Frontiers in pediatrics · 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
- Diagnostic Challenges in Pediatric Fever of Unknown Origin: Combined Role of Ferritin and Fever Duration.Children (Basel, Switzerland) · 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
- Still's disease: bridging the gap between guidelines and clinical practice.Pediatric rheumatology online journal · 2025Article
- Characteristics of 21 Patients with Secondary Hemophagocytic Lymphohistiocytosis-Insights from a Single-Center Retrospective Study.Medicina (Kaunas, Lithuania) · 2025Article
- Induction of LY6E regulates interleukin-1β production, potentially contributing to the immunopathogenesis of systemic lupus erythematosus.Cell communication and signaling : CCS · 2025Article
- T-ing up the storm: pathogenic cycling lymphocytes in the biology of macrophage activation syndrome.Pediatric rheumatology online journal · 2025Review
- Macrophage Activation Syndrome in Kawasaki Disease: Insights from a Systematic Literature Review on Diagnosis, Clinical Features, and Treatment.Children (Basel, Switzerland) · 2025Review
Corrections and comments
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Authors and funding
31 authors.
Funding
Abstract
objectiveTo assess current treatment in macrophage activation syndrome (MAS) worldwide and to highlight any areas of major heterogeneity of practice.
methodsA systematic literature search was performed in both EMBASE and PubMed databases. Paper screening was done by two independent teams based on agreed criteria. Data extraction was standardized following the PICO framework. A panel of experts assessed paper validity, using the Joanna Briggs Institute appraisal tools and category of evidence (CoE) according to EULAR procedure.
resultsFifty-seven papers were finally included (80% retrospective case-series), describing 1148 patients with MAS: 889 systemic juvenile idiopathic arthritis (sJIA), 137 systemic lupus erythematosus (SLE), 69 Kawasaki disease (KD) and 53 other rheumatological conditions. Fourteen and 11 studies specified data on MAS associated to SLE and KD, respectively. All papers mentioned glucocorticoids (GCs), mostly methylprednisolone and prednisolone (90%); dexamethasone was used in 7% of patients. Ciclosporin was reported in a wide range of patients according to different cohorts. Anakinra was used in 179 MAS patients, with a favourable outcome in 83% of sJIA-MAS. Etoposide was described by 11 studies, mainly as part of HLH-94/04 protocol. Emapalumab was the only medication tested in a clinical trial in 14 sJIA-MAS, with 93% of MAS remission. Ruxolitinib was the most reported Janus kinase inhibitor in MAS.
conclusionHigh-dose GCs together with IL-1 and IFNγ inhibitors have shown efficacy in MAS, especially in sJIA-associated MAS. However, the global level of evidence on MAS treatment, especially in other conditions, is still poor and requires standardized studies to be confirmed.
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Registered trials
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.