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.
Highlights
Kawasaki disease (KD), known as mucocutaneous lymph node syndrome, is one of the most common forms of vasculitis in children [1]
macrophage activation syndrome (MAS) is a type of hemophagocytic lymphohistiocytosis (HLH) that occurs under the circumstance of rheumatic diseases and is a life-threatening excessive immune activation syndrome
This report retrospectively analyzes three KD patients complicated with MAS who were treated in the pediatric intensive care unit (PICU) of our hospital and reviews the relevant literature in order to further our understanding of this disease and make an earlier diagnosis of this complication to improve prognosis through timely treatment
Summary
Kawasaki disease (KD), known as mucocutaneous lymph node syndrome, is one of the most common forms of vasculitis in children [1]. At the 3-month clinic follow-up visit, the repeat cardiac ultrasound showed a normal origin of LMCA at 2.3 mm (Z score + 1.05) and RCA at 1.6 mm (Z-score +0.14) in diameter This is a 2 year and 6-month-old boy, who presents to us for a cough that has lasted more than half a month and a recurrent fever 4 days. Cardiac ultrasound at the 3-month follow-up showed LMCA at 2.50 mm (Z-score +1.72) and RCA at 1.92 mm (Z-score +1.08) This case is a 5 year and 1-month old boy who presented with a history of fever for 5 days and sudden unconsciousness for 2 min (spontaneously recovered afterwards). The patient received a second dose of IVIG (2 g/kg/d) and 3 days of IV methylprednisolone (30 mg/kg/d), followed with improvement of symptoms and resolution of fever His cardiac ultrasound at the 3-month followup visit showed LMCA at 2.26 mm (Z-score +0.77) and RCA at 2.02 mm (Z-score +1.01)
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