SynthesisFrontiers in medicine2021
Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic Aspects.
Synthesis in Frontiers in medicine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers, 1 of them a synthesis that pooled it.
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Who cites it
15 citing papers in PubMed, 1 synthesis or guideline pooled it, 28 citations in OpenAlex.
- Eighty-six cases of clinical characteristics and outcomes of systemic lupus erythematosus-associated macrophage activation syndrome: A meta-analysis study.Immunity, inflammation and disease · 2024Pooled it
- Early management of hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a systematic literature review.EULAR rheumatology open · 2026Review
- A case report and literature review of neuropsychiatric lupus presenting with coma as the initial symptom.Medicine · 2026Review
- From Nerve to Autoimmunity: Acute Guillain-Barré Syndrome in a 4-Year-Old with Early-Onset Pediatric Systemic Lupus Erythematosus.Open access rheumatology : research and reviews · 2026Article
- A Study on Semaphorin 3A Level in Juvenile-onset Systemic Lupus Erythematosus Patients.Current rheumatology reviews · 2025Article
- Macrophage activation syndrome in Sepsis: from pathogenesis to clinical management.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2024Review
- BCD020 rituximab bioanalog compared to standard treatment in juvenile systemic lupus erythematosus: The data of 12 months case-control study.World journal of clinical pediatrics · 2024Article
- Stevens-Johnson syndrome and COVID-19: a case report with suspected multisystem inflammatory syndrome in children (MIS-C).Annals of medicine and surgery (2012) · 2023Article
- Blood Composite Scores in Patients with Systemic Lupus Erythematosus.Biomedicines · 2023Article
- Secondary haemophagocytic lymphohistiocytosis in a patient with new-onset systemic lupus erythematosus: the challenges of timely diagnosis and successful treatment.BMJ case reports · 2023Article
- Systemic lupus erythematosus with multicentric reticulohistiocytosis: A case report.World journal of clinical cases · 2023Article
- Massive Right Chylothorax Secondary to a Severe Systemic Lupus Erythematosus Flare With Secondary Evans Syndrome: A Case Report and Literature Review.Clinical medicine insights. Case reports · 2023Article
- Advances in attractive therapeutic approach for macrophage activation syndrome in COVID-19.Frontiers in immunology · 2023Review
- Strategy and Challenges of Paraclinical Examinations in Adult-Onset Still's Disease.Journal of clinical medicine · 2022Review
- Celiac Disease in Juvenile Idiopathic Arthritis and Other Pediatric Rheumatic Disorders.Journal of clinical medicine · 2022Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
6 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Macrophage Activation Syndrome (MAS) is a very severe complication of different rheumatic diseases, including pediatric Systemic Lupus Erythematosus (pSLE). MAS is not considered as a frequent complication of pSLE; however, its occurrence could be under-estimated and the diagnosis can be challenging. In order to address this issue, we performed a systematic review of the available medical literature, aiming to retrieve all those papers providing diagnostic (clinical/laboratory) data on patients with pSLE-related MAS, in individual or aggregated form. The selected case reports and series provided a pool of 46 patients, accounting for 48 episodes of MAS in total. We re-analyzed these patients in light of the diagnostic criteria for MAS validated in systemic Juvenile Idiopathic Arthritis (sJIA) patients and the preliminary diagnostic criteria for MAS in pSLE, respectively. Five clinical studies were also selected and used to support this analysis. This systematic review confirms that MAS diagnosis in pSLE patients is characterized by several diagnostic challenges, which could lead to delayed diagnosis and/or under-estimation of this complication. Specific criteria should be considered to diagnose MAS in different rheumatic diseases; as regards pSLE, the aforementioned preliminary criteria for MAS in pSLE seem to perform better than the sJIA-related MAS criteria, because of a lower ferritin cut-off.
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