ArticleFrontiers in pediatrics2022
Standard and increased canakinumab dosing to quiet macrophage activation syndrome in children with systemic juvenile idiopathic arthritis.
Article in Frontiers in pediatrics, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers, 2 of them syntheses that pooled it.
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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.
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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
18 citing papers in PubMed, 2 syntheses or guidelines pooled it, 21 citations in OpenAlex.
- Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project.Rheumatology (Oxford, England) · 2025Pooled it
- 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
- 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
- Cytokine storm.Nature reviews. Disease primers · 2026Review
- Dose Exploration Based on Quantitative Pharmacokinetic Analysis of Monoclonal Antibodies for Pediatric Immune-Mediated Diseases: Current Evidence and Future Directions.Drug design, development and therapy · 2026Review
- Evaluation of the real-world safety of Canakinumab in the treatment of systemic juvenile idiopathic arthritis in children aged 0-16: a comprehensive analysis based on FAERS data.Pediatric rheumatology online journal · 2025Article
- Low-dose emapalumab treatment in refractory macrophage activation syndrome secondary to adult onset still's disease/systemic lupus erythematosus: insights from nine cases.Frontiers in immunology · 2025Article
- Modulation of IRAK4 as a therapeutic strategy against monosodium urate- and xanthine-induced inflammation in macrophages and HepG2 cells.Frontiers in immunology · 2025Article
- Pharmacological and non-pharmacological treatments for refractory paediatric Still's disease: a scoping review.Rheumatology advances in practice · 2025Review
- Macrophage activation syndrome in Sepsis: from pathogenesis to clinical management.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2024Review
- Efficacy and safety of canakinumab in systemic juvenile idiopathic arthritis, the first Chinese experience.Pediatric rheumatology online journal · 2024Article
- Cytokines in Cytokine Storm Syndrome.Advances in experimental medicine and biology · 2024Review
- Canakinumab in the treatment of systemic juvenile idiopathic arthritis: a retrospective single center study in China.Frontiers in pediatrics · 2024Article
- Cytokine Storm Syndrome Associated with Systemic Juvenile Idiopathic Arthritis.Advances in experimental medicine and biology · 2024Review
- Systemic Lupus Erythematosus and Cytokine Storm.Advances in experimental medicine and biology · 2024Review
- Safety and efficacy of canakinumab treatment for undifferentiated autoinflammatory diseases: the data of a retrospective cohort two-centered study.Frontiers in medicine · 2023Article
- Advances in attractive therapeutic approach for macrophage activation syndrome in COVID-19.Frontiers in immunology · 2023Review
- Biomarkers in Systemic Juvenile Idiopathic Arthritis, Macrophage Activation Syndrome and Their Importance in COVID Era.International journal of molecular sciences · 2022Review
Corrections and comments
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Authors and funding
12 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Objective: Macrophage activation syndrome (MAS) is a life-threatening, potentially fatal condition associated with systemic juvenile idiopathic arthritis (sJIA). Interleukin-1 (IL-1) is a key cytokine in the pathogenesis of sJIA MAS. Many cases of MAS are medically refractory to traditional doses of biologic cytokine inhibitors and may require increased dosing. When MAS occurs in the setting of sJIA treated with the IL-1 receptor antagonist (IL-1Ra), anakinra, increased anakinra dosing may be beneficial. Increased dosing of another IL-1 inhibitor, canakinumab, a monoclonal antibody to IL-1β, has not been reported to treat refractory MAS in the setting of sJIA. Methods: Retrospective data collection extracted from the electronic medical record focused on canakinumab usage and dosing in 8 children with sJIA who developed MAS at a single academic center from 2011 to 2020. Results: Eight sJIA children (five girls) with median age 8.5 years (range, 0.9-14.2 years) were included in the present study. Five children developed MAS at disease onset and three during ongoing canakinumab therapy. MAS resolved in all eight children with canakinumab treatment. When the canakinumab dosing was insufficient or MAS developed during canakinumab therapy, the dosing was temporally up-titrated (four patients, maximum 300 mg per dose) without observed side effects. Conclusion: This report provides evidence for the efficacy and safety of short-term increased doses (2-3-times normal) of canakinumab in treating sJIA associated MAS. Further study of the efficacy and safety of increased doses of canakinumab for treatment of MAS in children with sJIA is warranted.
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