Macrophage activation syndrome: A challenge for pediatrician from rheumatology department and pediatric intensive care unit
10.3760/cma.j.issn.1673-4912.2020.05.003
- VernacularTitle:巨噬细胞活化综合征：儿童风湿科及PICU医师的共同挑战
- Author:
Haiguo YU
1
Author Information
1. 南京医科大学附属儿童医院风湿免疫科 210008
- From:
Chinese Pediatric Emergency Medicine
2020;27(5):330-335
- CountryChina
- Language:Chinese
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Abstract:
Macrophage activation syndrome(MAS)is a potentially life-threatening complication of rheumatic disorders.Macrophage activation-like syndrome refers to sepsis patient with dominant pro-inflammatory mechanism of organ dysfunction.Decreased cytolytic function results in prolonged interaction of the effective lymphocytes and their target antigen presenting cells, thus resulting in the pro-inflammatory cytokine storm which lead to multi-organ systerm failure.Early diagnosis remains challenging even there is a set of universal diagnostic criteria.Intravenous pulse methylprednisolon combination with cyclosporin A is still the mainstream therapeutic protocol in domestic.Novel cytokine-targeted approaches are being explored for safer and effective therapies for children with MAS.
