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Updated: Mar 1, 2026
Whole-cell MALDI-TOF Mass Spectrometry is an Accurate and Rapid Method to Analyze Different Modes of Macrophage Activation
Published on: December 26, 2013
Kawasaki Disease Complicated With Macrophage Activation Syndrome: A Systematic Review
Susana García-Pavón1, Marco A Yamazaki-Nakashimada, Milton Báez
1*Allergy and Immunology Department, Naval Hospital of High Speciality †Clinical Immunology Department, National Institute of Pediatrics ‡Pediatric Private Practice §Research Methodology Department, National Institute of Pediatrics.
Abstract:
Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis, is a rare and potentially fatal complication of Kawasaki disease (KD). We report 2 cases, performed a literature search, and analyze the characteristics of MAS associated with KD. A total of 69 patients were evaluated, 34 reported the date of the diagnosis of MAS and KD, 6% had a diagnosis of MAS before KD, 21% had a simultaneous presentation, and 73% had the diagnosis of MAS after KD. Different treatment approaches were observed with corticosteroids administered in 87%, cyclosporine in 49%, etoposide (VP-16) in 39%, and monoclonal anti-TNF in 6% of cases. Coronary abnormalities were especially high in this group of patients (46%) and 9 patients died (13%). The persistence of fever with splenomegaly, hyperferritinemia, thrombocytopenia, and elevated aspartate aminotransferase (AST) should prompt the consideration of MAS complicating KD.
Insights
Macrophage activation syndrome (MAS), a severe complication of Kawasaki disease (KD), presents unique challenges. Early recognition of MAS in KD patients is crucial for timely intervention and improved outcomes.
Area of Science:
- Pediatrics
- Rheumatology
- Hematology
Background:
- Macrophage activation syndrome (MAS), or secondary hemophagocytic lymphohistiocytosis, is a rare but life-threatening complication of Kawasaki disease (KD).
- Understanding the characteristics and outcomes of MAS in KD is essential for clinical management.
Purpose of the Study:
- To analyze the characteristics of MAS associated with KD.
- To review treatment approaches and outcomes in patients with KD-associated MAS.
Main Methods:
- Case report of 2 patients with MAS complicating KD.
- Comprehensive literature search and analysis of 69 patients with KD-associated MAS.
- Evaluation of diagnostic timing, treatment modalities, and clinical outcomes.
Main Results:
- MAS diagnosis occurred after KD in 73% of patients, simultaneously in 21%, and before KD in 6%.
- Corticosteroids were used in 87%, cyclosporine in 49%, etoposide (VP-16) in 39%, and anti-TNF agents in 6%.
- High rates of coronary abnormalities (46%) and mortality (13%) were observed in this cohort.
Conclusions:
- Persistent fever, splenomegaly, hyperferritinemia, thrombocytopenia, and elevated AST warrant consideration of MAS in KD.
- Prompt diagnosis and appropriate management of MAS in KD are critical to reduce morbidity and mortality.
