ArticleRheumatology advances in practice2019
Clinical features of macrophage activation syndrome as the onset manifestation of juvenile systemic lupus erythematosus.
Article in Rheumatology advances in practice, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers, 1 of them a synthesis that pooled it.
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Who cites it
11 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic Aspects.Frontiers in medicine · 2021Pooled it
- 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
- Childhood-Onset Systemic Lupus Erythematosus Research over the Past Decade in Japan.Children (Basel, Switzerland) · 2026Review
- Systemic Lupus Erythematosus and Lung Involvement: A Comprehensive Review.Journal of clinical medicine · 2022Review
- Article
- Pathophysiologic mechanisms of cerebral endotheliopathy and stroke due to Sars-CoV-2.Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism · 2021Review
- Macrophage activation syndrome in systemic lupus erythematosus and systemic-onset juvenile idiopathic arthritis: a retrospective study of similarities and dissimilarities.Rheumatology international · 2021Observational
- Multisystem Inflammatory Syndrome in Children Associated with Status Epilepticus.The Journal of pediatrics · 2020Article
- Case of using mycophenolate in combination with steroids for concurrent macrophage activation syndrome and lupus flare.BMJ case reports · 2020Article
- Macrophage activation syndrome as an initial presentation of systemic lupus erythematosus with sensorineural hearing loss in a young male patient.BMJ case reports · 2020Article
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8 authors.
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Abstract
objectivesMacrophage activation syndrome (MAS) is a severe complication of juvenile systemic lupus erythematosus (jSLE). However, little is known about the association between these conditions, especially in terms of MAS as the initial manifestation of jSLE. The aim of this study was to determine the clinical features of MAS as the initial manifestation of jSLE.
methodsWe carried out a retrospective review of the clinical features of MAS cases diagnosed concomitantly with jSLE from 2004 to 2016. Data from these patients were compared with those from a control group consisting of jSLE patients without MAS.
resultsEleven (23.9%) of the 46 patients recruited for this study were diagnosed with MAS during the initial stage of jSLE. The between-group comparisons demonstrated that fever, leucopenia, hyperferritinaemia and increased aspartate aminotransferase were more frequently observed in jSLE patients with MAS than in controls (
conclusionMAS can develop as the initial manifestation of jSLE. MAS with jSLE should be suspected in patients with fever, hyperferritinaemia, cytopenia and liver disorder. In addition, we found that jSLE patients with MAS had more neurological symptoms than those without. All patients with MAS were successfully treated with CSs. Early diagnosis and intensive therapy are essential in improving clinical outcomes.
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