ReviewPediatric rheumatology online journal2017
Macrophage Activation Syndrome: different mechanisms leading to a one clinical syndrome.
Review in Pediatric rheumatology online journal, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 88 papers, 4 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
88 citing papers in PubMed, 4 syntheses or guidelines pooled it, 164 citations in OpenAlex.
- 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.Annals of the rheumatic diseases · 2024Pooled it
- Immunopathology of and potential therapeutics for secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a translational perspective.Experimental & molecular medicine · 2024Pooled it
- An Integrative Transcriptomic Analysis of Systemic Juvenile Idiopathic Arthritis for Identifying Potential Genetic Markers and Drug Candidates.International journal of molecular sciences · 2021Pooled it
- Macrophage activation syndrome in juvenile dermatomyositis: a systematic review.Rheumatology international · 2020Pooled it
- Efficacy and safety of acalabrutinib with best supportive care versus best supportive care in patients with COVID-19 requiring hospitalization.ImmunoHorizons · 2025Trial
- Critical care outcomes of hemophagocytic lymphohistiocytosis in children: a single centre retrospective observational study.Pediatric rheumatology online journal · 2026Observational
- Early management of hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a systematic literature review.EULAR rheumatology open · 2026Review
- Clinical characteristics and diagnostic indicators of childhood-onset systemic lupus erythematosus complicated with macrophage activation syndrome.Clinical rheumatology · 2026Article
- Decoy Receptors in Autoimmunity: Molecular Guardians and Pathogenic Players in Immune Dysregulation.Mediators of inflammation · 2026Review
- Innate Lymphoid Cell Phenotypic and Functional Alterations in Patients With Systemic Juvenile Idiopathic Arthritis.Arthritis & rheumatology (Hoboken, N.J.) · 2025Article
- Clinical and genetic analysis of macrophage activation syndrome complicating juvenile idiopathic inflammatory myopathies.Pediatric research · 2025Article
- Temperature-Dependent Cytokine Neutralization Induced by Magnetoelectric Nanoparticles: An In Silico Study.International journal of molecular sciences · 2024Article
- New discoveries in the genetics and genomics of systemic juvenile idiopathic arthritis.Expert review of clinical immunology · 2024Review
- Hemophagocytic Lymphohistiocytosis Triggered by Herpes Simplex Virus 1 and 2: A Narrative Review.Hematology reports · 2024Review
- From multisystem inflammatory syndrome in children to secondary hemophagocytic lymphohistiocytosis a series of misfortunate events: case report and review of literature.Sudanese journal of paediatrics · 2024Article
- Tmem178 Negatively Regulates IL-1β Production Through Inhibition of the NLRP3 Inflammasome.Arthritis & rheumatology (Hoboken, N.J.) · 2024Article
- Cytokine Storm Syndrome Associated with Systemic Juvenile Idiopathic Arthritis.Advances in experimental medicine and biology · 2024Review
- Immunology of Cytokine Storm Syndromes: Natural Killer Cells.Advances in experimental medicine and biology · 2024Review
- Immune Mechanisms in Inflammatory Anemia.Annual review of immunology · 2023Review
- Post-COVID-19 syndrome: Insights into a novel post-infectious systemic disorder.Journal of medicine and life · 2023Review
28 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
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundMacrophage activation syndrome (MAS) is a severe complication of rheumatic disease in childhood, particularly in systemic Juvenile Idiopathic Arthritis (sJIA). It is characterize by an uncontrolled activation and proliferation of T lymphocytes and macrophages. MAIN CONTENT: MAS is currently classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH). The reason is that MAS shares clinical and laboratory features with primary genetic HLH (pHLH). In this context is conceivable that some of the pathogenic mechanisms of pHLH may be involved in other forms of HLH. Heterozygosity for mutations of genes involved in pHLH may lead to a cytotoxic defect and to a development of clinical overt disease. But other different contributors might be involved to the development of MAS such as infections or underlying inflammation. In MAS, the inflammatory status of the patient is a major contributor of the disease. Indeed, the majority of the MAS episodes occurs during active disease phases or at disease onset. In addition, recent evidence in animals and humans suggest that genetics may also play a major role in contributing to hyperinflammation and particularly to macrophages hyper-responses.
conclusionsWe hypothesize that HLH may be one unique clinical syndrome, to whose generation different mechanisms may contribute, and maintained by one final effector mechanism.
Indexed as
Identifiers
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.