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Kawasaki Disease Complicated With Macrophage Activation Syndrome: Case Reports and Literature Review
1Pediatric Intensive Care Unit, Baoan Maternal and Child Health Hospital, College of Medicine, Jinan University, Shenzhen, China.
Abstract:
Macrophage activation syndrome (MAS) is a rare and severe complication of Kawasaki disease (KD). The clinical feature, early diagnosis and treatment options, and prognosis need to be further determined in patients with KD complicated with MAS. In this report, we retrospectively analyzed three KD patients complicated with MAS who were treated in pediatric intensive care units (PICU) and reviewed the relevant literatures. We find that being male, being age over 2 years old, incomplete KD, intravenous immunoglobulin (IVIG) non-responder, or persistent fever greater than 10 days are all highly associated with occurrence of MAS. Additional work-ups should be performed promptly in patient with above predisposing factors to rule out complication of MAS. Patients with KD complicated with MAS are at a higher risk of having coronary artery involvement or aneurysm formation, which can be reversed with timely treatment. Early identification and prompt treatment are key points for improving the prognosis of KD patients complicated with MAS.
Insights
Macrophage activation syndrome (MAS) is a severe Kawasaki disease (KD) complication. Early identification and prompt treatment of MAS in KD patients improve outcomes and reduce coronary artery risks.
Area of Science:
- Pediatric Rheumatology
- Intensive Care Medicine
Background:
- Macrophage activation syndrome (MAS) is a rare but severe complication of Kawasaki disease (KD).
- Understanding the clinical features, diagnosis, and treatment of MAS in KD patients is crucial for improving prognosis.
Observation:
- This study retrospectively analyzed three pediatric intensive care unit (PICU) patients with KD complicated by MAS.
- Literature review was conducted to further elucidate the condition.
Findings:
- Male gender, age over 2 years, incomplete KD, non-response to intravenous immunoglobulin (IVIG), and persistent fever (>10 days) are risk factors for MAS in KD.
- Patients with KD and MAS face increased risk of coronary artery abnormalities, which are reversible with timely intervention.
Implications:
- Prompt work-ups for MAS are recommended in KD patients with predisposing factors.
- Early recognition and treatment are vital for improving the prognosis of KD patients with MAS, particularly regarding cardiac complications.
