SynthesisPediatric rheumatology online journal2015
Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis.
Synthesis in Pediatric rheumatology online journal, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 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
38 citing papers in PubMed, 2 syntheses or guidelines pooled it, 96 citations in OpenAlex.
- Risk factors for macrophage activation syndrome in systemic juvenile idiopathic arthritis: a systematic review and meta-analysis.Frontiers in pediatrics · 2025Pooled it
- Anakinra treatment in macrophage activation syndrome: a single center experience and systemic review of literature.Clinical rheumatology · 2018Pooled it
- Adults with septic shock and extreme hyperferritinemia exhibit pathogenic immune variation.Genes and immunity · 2019Trial
- African recommendations for diagnosis and management of systemic juvenile idiopathic arthritis (sJIA) - a PAFLAR initiative.Pediatric rheumatology online journal · 2026Article
- T-ing up the storm: pathogenic cycling lymphocytes in the biology of macrophage activation syndrome.Pediatric rheumatology online journal · 2025Review
- Tmem178 Negatively Regulates IL-1β Production Through Inhibition of the NLRP3 Inflammasome.Arthritis & rheumatology (Hoboken, N.J.) · 2024Article
- Other Immunomodulatory Treatment for Cytokine Storm Syndromes.Advances in experimental medicine and biology · 2024Review
- Macrophage activation syndrome in juvenile dermatomyositis: a case report and a comprehensive review of the literature.Pediatric rheumatology online journal · 2023Review
- Efficacy and safety of emapalumab in macrophage activation syndrome.Annals of the rheumatic diseases · 2023Article
- Systemic Juvenile Idiopathic Arthritis and Secondary Macrophage Activation Syndrome in Latvia from 2009 to 2020: A Nationwide Retrospective Study.Medicina (Kaunas, Lithuania) · 2023Article
- Case report: Suspected transfusion-related acute lung injury type II in a child with refractory systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome.Frontiers in pediatrics · 2023Article
- Hypersensitivity to Biological Treatments in Juvenile Idiopathic Arthritis: How Should It Be Managed?Journal of clinical medicine · 2022Review
- Refractory systemic onset juvenile idiopathic arthritis: current challenges and future perspectives.Annals of medicine · 2022Review
- Implications of SARS-CoV-2 Infection in Systemic Juvenile Idiopathic Arthritis.International journal of molecular sciences · 2022Review
- Systemic Juvenile Idiopathic Arthritis/Pediatric Still's Disease, a Syndrome but Several Clinical Forms: Recent Therapeutic Approaches.Journal of clinical medicine · 2022Article
- Juvenile idiopathic arthritis.Nature reviews. Disease primers · 2022Review
- Rare complications of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis: Time to nip them in the bud.Frontiers in immunology · 2022Review
- Macrophage activation syndrome in adults: Characteristics, outcomes, and therapeutic effectiveness of etoposide-based regimen.Frontiers in immunology · 2022Article
- B Cells on the Stage of Inflammation in Juvenile Idiopathic Arthritis: Leading or Supporting Actors in Disease Pathogenesis?Frontiers in medicine · 2022Review
- Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis complicated with macrophage activation syndrome.Therapeutic advances in chronic disease · 2022Article
Corrections and comments
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Authors and funding
7 authors at 6 institutions in 5 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundMacrophage activation syndrome (MAS) is a severe and potentially lethal complication of several inflammatory diseases but seems particularly linked to systemic juvenile idiopathic arthritis (sJIA). Standardized diagnostic and treatment guidelines for MAS in sJIA are currently lacking. The aim of this systematic literature review was to evaluate currently available literature on diagnostic criteria for MAS in sJIA and provide an overview of possible biomarkers for diagnosis, disease activity and treatment response and recent advances in treatment.
methodsA systematic literature search was performed in MEDLINE, EMBASE and Cochrane. 495 papers were identified. Potentially relevant papers were selected by 3 authors after which full text screening was performed. All selected papers were evaluated by at least two independent experts for validity and level of evidence according to EULAR guidelines.
results27 papers were included: 7 on diagnosis, 9 on biomarkers and 11 on treatment. Systematic review of the literature confirmed that there are no validated diagnostic criteria for MAS in sJIA. The preliminary Ravelli criteria, with the addition of ferritin, performed well in a large retrospective case-control study. Recently, an international consortium lead by PRINTO proposed a new set of diagnostic criteria able to distinguish MAS from active sJIA and/or infection with superior performance. Other promising diagnostic biomarkers potentially distinguish MAS complicating sJIA from primary and virus-associated hemophagocytic lymphohistiocytosis. The highest level of evidence for treatment comes from case-series. High dose corticosteroids with or without cyclosporine A were frequently reported as first-line therapy. From the newer treatment modalities, promising responses have been reported with anakinra.
conclusionMAS in sJIA seems to be diagnosed best by the recently proposed PRINTO criteria, although prospective validation is needed. Novel promising biomarkers for sJIA related MAS are in need of prospective validation as well, and are not widely available yet. Currently, treatment of MAS in sJIA relies more on experience than evidence based medicine. Taking into account the severity of MAS and the scarcity of evidence, early expert consultation is recommended as soon as MAS is suspected.
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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.