ArticleRheumatology international2016
Macrophage activation syndrome in children with systemic juvenile idiopathic arthritis and systemic lupus erythematosus.
Article in Rheumatology international, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers, 6 of them syntheses that pooled it.
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Who cites it
38 citing papers in PubMed, 6 syntheses or guidelines pooled it, 60 citations in OpenAlex.
- Guidelines on the Use of Therapeutic Apheresis in Clinical Practice-Evidence-Based Approach From the Writing Committee of the American Society for Apheresis: The Tenth Special Issue.Journal of clinical apheresis · 2026Guideline
- Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project.Rheumatology (Oxford, England) · 2025Pooled it
- 2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis.Arthritis care & research · 2022Guideline
- 2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis.Arthritis & rheumatology (Hoboken, N.J.) · 2022Guideline
- Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic Aspects.Frontiers in medicine · 2021Pooled it
- Anakinra treatment in macrophage activation syndrome: a single center experience and systemic review of literature.Clinical rheumatology · 2018Pooled it
- Clinical characteristics and diagnostic indicators of childhood-onset systemic lupus erythematosus complicated with macrophage activation syndrome.Clinical rheumatology · 2026Article
- A MASsive attack: a pediatric case of macrophage activation syndrome complicated by DIC as an onset of systemic juvenile idiopathic arthritis successfully treated with anakinra and review of the literature.Rheumatology international · 2024Review
- Epstein-Barr Virus encephalitis associated hemophagocytic lymphohistiocytosis in childhood-onset systemic lupus erythematosus: a case-based review.Pediatric rheumatology online journal · 2024Review
- The NLRP3 inflammasome is essential for IL-18 production in a murine model of macrophage activation syndrome.Disease models & mechanisms · 2024Article
- The NLRP3 inflammasome is essential for IL-18 production in a murine model of macrophage activation syndrome.bioRxiv : the preprint server for biology · 2024Article
- Is it possible to predict a disease course prone to macrophage activation syndrome at systemic juvenile idiopathic arthritis diagnosis?Clinical rheumatology · 2024Article
- Clinical Features of Cytokine Storm Syndrome.Advances in experimental medicine and biology · 2024Review
- The History of Macrophage Activation Syndrome in Autoimmune Diseases.Advances in experimental medicine and biology · 2024Review
- Systemic Lupus Erythematosus and Cytokine Storm.Advances in experimental medicine and biology · 2024Review
- IL-1 Family Blockade in Cytokine Storm Syndromes.Advances in experimental medicine and biology · 2024Review
- Other Immunomodulatory Treatment for Cytokine Storm Syndromes.Advances in experimental medicine and biology · 2024Review
- Increased ferritin, serum lactate dehydrogenase, and aspartate aminotransferase levels predict macrophage activation syndrome complicating systemic lupus erythematosus: a retrospective study.Frontiers in pediatrics · 2024Article
- Heterogeneity of macrophage activation syndrome and treatment progression.Frontiers in immunology · 2024Review
- Lung Involvement in Systemic Juvenile Idiopathic Arthritis: A Narrative Review.Diagnostics (Basel, Switzerland) · 2022Review
Corrections and comments
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Macrophage activation syndrome (MAS) is a hyper-inflammatory disorder secondary to a rheumatic disease such as systemic juvenile idiopathic arthritis (SJIA) and systemic lupus erythematosus (SLE). We aimed to present the characteristics of our pediatric MAS patients. Clinical features, laboratory parameters, treatment, and outcome of 34 patients (28 SJIA; six SLE; 37 MAS episodes) followed at a tertiary health center between 2009 and 2015 were retrospectively reviewed. The median age at MAS onset was 11 years. More SJIA patients had MAS at disease onset than SLE patients (53.6 vs. 16.7 %). Fever, high C-reactive protein and hyperferritinemia were present in all MAS episodes. Rash was less (p = 0.03), and fatigue was more frequent (p = 0.042) in SLE than SJIA patients. All received corticosteroids. Cyclosporine was given in 74.2 % of SJIA-MAS; 66.7 % of SLE-MAS episodes. Intravenous immunoglobulin, anakinra, or etoposide was administered during 67.7; 41.9; 32.3 % of SJIA-MAS and 33.3; 33.3; 50 % of SLE-MAS episodes, respectively. Plasmapheresis was performed during 41.9 % of SJIA-MAS and 33.3 % of SLE-MAS episodes. The mortality rate was 11.8 % (n = 4;3 SJIA, 1 SLE). Hepatosplenomegaly was more frequent (p = 0.005), and plasmapheresis was performed more frequently (p = 0.021) in the patients who died compared to the cured patients. The median duration between symptom onset and admission to our hospital was longer among the patients who died (16.5 vs. 7 days; p = 0.049). Our patients' characteristics were similar to the reported cases, but our mortality rate is slightly higher probably due to late referral to our center. Early diagnosis and effective treatment are crucial to prevent mortality.
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