ReviewGenes and immunity2012
Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment.
Review in Genes and immunity, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 168 papers, 2 of them syntheses that pooled it.
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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
168 citing papers in PubMed, 2 syntheses or guidelines pooled it, 380 citations in OpenAlex.
- The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS).Arthritis & rheumatology (Hoboken, N.J.) · 2023Pooled it
- The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS).Annals of the rheumatic diseases · 2023Pooled it
- Adults with septic shock and extreme hyperferritinemia exhibit pathogenic immune variation.Genes and immunity · 2019Trial
- Interleukin-1 Receptor Blockade Is Associated With Reduced Mortality in Sepsis Patients With Features of Macrophage Activation Syndrome: Reanalysis of a Prior Phase III Trial.Critical care medicine · 2016Trial
- Gene-expression analysis of adult-onset Still's disease and systemic juvenile idiopathic arthritis is consistent with a continuum of a single disease entity.Pediatric rheumatology online journal · 2015Trial
- Lipid and immune profiles in children with systemic juvenile idiopathic arthritis: a case-control study with paired longitudinal comparison of disease activity.Translational pediatrics · 2026Article
- Macrophage activation syndrome in systemic lupus erythematous and Still's disease: distinct phenotypes shaped by the underlying diseases.Rheumatology (Oxford, England) · 2026Article
- Critical care outcomes of hemophagocytic lymphohistiocytosis in children: a single centre retrospective observational study.Pediatric rheumatology online journal · 2026Observational
- Chimeric antigen receptor macrophage (CAR-M) immunotherapy in autoimmune inflammatory rheumatic diseases.Clinical rheumatology · 2026Review
- Enhancing the precision of clinical assessment in juvenile idiopathic arthritis through the JADAS.Pediatric rheumatology online journal · 2026Review
- Severe Adult HLH/MAS With SPTCL-Like Panniculitis: A Phenotype-Guided, Resource-Adapted Therapeutic Strategy Without Cytotoxic Therapy.Clinical case reports · 2026Article
- Clinical characteristics and diagnostic indicators of childhood-onset systemic lupus erythematosus complicated with macrophage activation syndrome.Clinical rheumatology · 2026Article
- Emergency in pediatric rheumatology: a narrative review.Frontiers in medicine · 2026Review
- Article
- Case Report: Ruxolitinib for systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome: two pediatric cases and literature review.Frontiers in pediatrics · 2026Article
- Emapalumab as a Life-Saving Therapy for Macrophage Activation Syndrome in Refractory Systemic Juvenile Idiopathic Arthritis with Trisomy 21.Turkish archives of pediatrics · 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
- Management of Steroid-Refractory Serositis and Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis.Indian journal of pediatrics · 2025Article
- Haemophagocytic lymphohistiocytosis in critical care.BJA education · 2025Review
108 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 4 institutions in 2 countries.
Funding
Abstract
Macrophage activation syndrome (MAS) is a severe, frequently fatal complication of systemic juvenile idiopathic arthritis (sJIA) with features of hemophagocytosis leading to coagulopathy, pancytopenia, and liver and central nervous system dysfunction. MAS is overt in 10% of children with sJIA but occurs subclinically in another 30-40%. It is difficult to distinguish sJIA disease flare from MAS. Development of criteria for establishing MAS as part of sJIA are under way and will hopefully prove sensitive and specific. Mutations in cytolytic pathway genes are increasingly being recognized in children who develop MAS as part of sJIA. Identification of these mutations may someday assist in MAS diagnosis. Defects in cytolytic genes have provided murine models of MAS to study pathophysiology and treatment. Recently, the first mouse model of MAS not requiring infection but rather dependent on repeated stimulation through Toll-like receptors was reported. This provides a model of MAS that may more accurately reflect MAS pathology in the setting of autoinflammation or autoimmunity. This model confirms the importance of a balance between pro- and anti-inflammatory cytokines. There has been remarkable progress in the use of anti-pro-inflammatory cytokine therapy, particularly against interleukin-1, in the treatment of secondary forms of MAS, such as in sJIA.
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What OpenQuestion holds
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.