ReviewPaediatric drugs2020
Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management.
Review in Paediatric drugs, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 90 papers, 3 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
90 citing papers in PubMed, 3 syntheses or guidelines pooled it, 157 citations in OpenAlex.
- 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease.Arthritis & rheumatology (Hoboken, N.J.) · 2022Guideline
- 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease.Arthritis care & research · 2022Guideline
- Ferritin in the coronavirus disease 2019 (COVID-19): A systematic review and meta-analysis.Journal of clinical laboratory analysis · 2020Pooled it
- Macrophage activation syndrome in a 13-month-old child with systemic juvenile idiopathic arthritis mimicking sepsis: a diagnostic challenge and case report.Annals of medicine and surgery (2012) · 2026Article
- Diagnostic performance of HScore for macrophage activation syndrome complicating Still's disease in adults: a multicentre case-control study.Rheumatology (Oxford, England) · 2026Article
- Effectiveness and Safety of Rituximab in Patients with Refractory Juvenile Idiopathic Arthritis: A Tertiary Center Experience.Pharmaceuticals (Basel, Switzerland) · 2026Article
- Evaluating the significance of platelet count, neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio in critically ill children.Scientific reports · 2026Article
- Cytokine storm.Nature reviews. Disease primers · 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
- Adult-Onset Still's Disease in a Patient with Macrophage Activation Syndrome and Pre-Disseminated Intravascular Coagulation: A Case Report and Literature Review.Journal of inflammation research · 2026Article
- Emergency in pediatric rheumatology: a narrative review.Frontiers in medicine · 2026Review
- Dynamic hyperinflammatory response assessment using HIC scores in COVID-19: application to a large series of patients receiving anakinra.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
- Intensive care unit admission and mortality in rheumatic diseases.Rheumatology international · 2025Review
- Article
- Hemophagocytic Lymphohistiocytosis Gene Variants in Severe COVID-19 Cytokine Storm Syndrome.Viruses · 2025Article
- Fatal Cytokine Collision: HLH-AIHA in Advanced AIDS-Case Report and Literature Review.Reports (MDPI) · 2025Article
- Rare early hematogenous disseminated tuberculosis inducing hemophagocytic syndrome in conflict treatment.BMC infectious diseases · 2025Article
- Article
- T-ing up the storm: pathogenic cycling lymphocytes in the biology of macrophage activation syndrome.Pediatric rheumatology online journal · 2025Review
30 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
2 authors at 2 institutions in 1 country.
Funding
Abstract
Macrophage activation syndrome (MAS), a form of secondary hemophagocytic lymphohistiocytosis, is a frequently fatal complication of a variety of pediatric inflammatory disorders. MAS has been most commonly associated with systemic juvenile idiopathic arthritis (sJIA), as approximately 10% of children with sJIA develop fulminant MAS, with another 30-40% exhibiting a more subclinical form of the disease. Children with other rheumatologic conditions such as systemic lupus erythematosus and Kawasaki disease are also at risk for MAS. Moreover, MAS also complicates various genetic autoinflammatory disorders such as gain of function mutations in the cytosolic inflammasome NLRC4, pediatric hematologic malignancies (e.g., T-cell lymphoma), and primary immunodeficiencies characterized by immune dysregulation. Disease-specific and broadly inclusive diagnostic criteria have been developed to facilitate the diagnosis of MAS. Recently, simple screening tools such as the serum ferritin to erythrocyte sedimentation rate ratio have been proposed. Early diagnosis and rapid initiation of immunosuppression are essential for the effective management of MAS. With a better understanding of the pathophysiology of MAS and the advent of novel therapeutics, a broad immunosuppressive approach to treatment is giving way to targeted anti-cytokine therapies. These treatments include agents that block interleukin-1 (IL-1), IL-6, IL-18, interferon-γ, as well as inhibitors of downstream targets of cytokine signaling (e.g., Janus kinases). Increased early recognition of MAS among pediatric inflammatory disorders combined with the use of effective and less toxic cytokine-targeted therapies should lower the mortality of this frequently fatal disorder.
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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.