ReviewWorld journal of pediatrics : WJP2020
Macrophage activation syndrome in children with Kawasaki disease: diagnostic and therapeutic approaches.
Review in World journal of pediatrics : WJP, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 47 citations in OpenAlex.
- 2016 sJIA-MAS criteria and a step-up therapeutic approach in KD-MAS: insights from a combined cohort and literature review.RMD open · 2026Review
- Incomplete Kawasaki disease associated with acute icteric hepatitis and Torque teno virus infection: a case report and literature review.BMC pediatrics · 2026Review
- Prediction nomogram of serum interferon-γ enable early identification of macrophage activation syndrome in children with Kawasaki disease.Clinical rheumatology · 2026Article
- Refractory Kawasaki Disease in Infants Complicated with Giant Coronary Artery Aneurysms and Takayasu Arteritis: A Rare Case Report and Literature Review.Vascular health and risk management · 2026Review
- Ruxolitinib combined with corticosteroids for the successful treatment of kawasaki disease-associated macrophage activation syndrome: a case report.Frontiers in pediatrics · 2026Article
- Clinical value of combined detection of serum ferritin and N-terminal pro-brain natriuretic peptide in the assessment of Kawasaki disease in children over 1 year of age.Italian journal of pediatrics · 2025Article
- Macrophage Activation Syndrome in Kawasaki Disease: Insights from a Systematic Literature Review on Diagnosis, Clinical Features, and Treatment.Children (Basel, Switzerland) · 2025Review
- Identifying the risk of Kawasaki disease based solely on routine blood test features through novel construction of machine learning models.Computational and structural biotechnology journal · 2025Article
- Ferroptosis-related oxidative stress activation in the acute phase of Kawasaki disease.Frontiers in immunology · 2025Article
- Development of Preliminary Criteria of Macrophage Activation Syndrome in Multisystem Inflammatory Syndrome Associated with COVID-19 in Children.Biomedicines · 2024Article
- Macrophage Activation Syndrome in Children: Update on Diagnosis and Treatment.Children (Basel, Switzerland) · 2024Review
- Single center clinical analysis of macrophage activation syndrome complicating juvenile rheumatic diseases.Pediatric rheumatology online journal · 2024Article
- Using HScore for Evaluation of Hemophagocytosis in Multisystem Inflammatory Syndrome Associated with COVID-19 in Children.Biomedicines · 2024Article
- Role of Pyroptosis in IVIG-Resistant Kawasaki Disease and the Establishment of a New Predictive Model.Journal of inflammation research · 2024Article
- Kawasaki Disease-Associated Cytokine Storm Syndrome.Advances in experimental medicine and biology · 2024Review
- Utilizing T-Lymphocyte Activation-Related Cytokines to Predict Non-Responsiveness to Treatment in Pediatric Kawasaki Disease.Pediatric health, medicine and therapeutics · 2024Article
- Clinical Signs of Kawasaki Disease from the Perspective of Epithelial-to-Mesenchymal Transition Recruiting Erythrocytes: A Literature Review.Reviews in cardiovascular medicine · 2023Review
- Kawasaki disease complicated with shock syndrome, macrophage activation syndrome, and acute abdomen in children: Two case reports.Frontiers in pediatrics · 2023Article
- Platelet and ferritin as early predictive factors for the development of macrophage activation syndrome in children with Kawasaki disease: A retrospective case-control study.Frontiers in pediatrics · 2023Article
- A novel model for predicting intravenous immunoglobulin-resistance in Kawasaki disease: a large cohort study.Frontiers in cardiovascular medicine · 2023Article
Corrections and comments
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Authors and funding
2 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundMacrophage activation syndrome (MAS) is a rare, life-threatening complication of Kawasaki disease (KD). Early recognition and treatment of MAS are very important, but sometimes it is difficult to distinguish MAS from a severe form of KD. DATA SOURCES: A PubMed search was performed in Clinical Queries using the key terms "macrophage activation syndrome or secondary hemophagocytic lymphohistiocytosis (HLH)" and "Kawasaki disease".
resultsKD patients with MAS show high intravenous immunoglobulin (IVIG) resistance and coronary complications. Mortality is also as high as MAS in other diseases. Persistent fever greater than 10 days is highly associated with development of MAS in KD. Splenomegaly is observed in more than two-thirds of KD patients with MAS. Thrombocytopenia is often the earliest laboratory finding of MAS. Hyperferritinemia is highly specific and sensitive for detecting MAS in KD; so, ferritin levels should be checked if there are unexplained clinical exacerbations in KD patients. Given the under-recognition of MAS in KD, it is prudent to consider resistant KD as occult/subclinical MAS. Many KD patients with MAS have good outcomes on immune modulators. However, if KD patients fulfill the HLH-2004 diagnostic criteria, they may undergo longer and more intensive treatment than needed.
conclusionsThe possible existence of MAS should be taken into account when a KD patient shows persistent fever, splenomegaly, thrombocytopenia, hyperferritinemia, or IVIG resistance. The under-diagnosis of MAS in patients with KD is an important issue to be addressed. Therapeutically, however, there is a possibility of over-treatment of MAS in patients with KD.
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Registered trials
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.