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Macrophage activation syndrome in the acute phase of Kawasaki disease

Giuseppe A Latino1, Cedric Manlhiot, Rae S M Yeung

  • 1Division of Cardiology, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.

Journal of Pediatric Hematology/Oncology
|May 21, 2010
PubMed

Insights

Rare cases of macrophage activation syndrome (MAS) in Kawasaki disease (KD) patients were studied. Prompt treatment beyond standard protocols led to full recovery without long-term effects, highlighting the need for early MAS detection in KD.

Area of Science:

  • Pediatrics
  • Rheumatology
  • Immunology

Background:

  • Macrophage activation syndrome (MAS) is a rare but severe complication.
  • Kawasaki disease (KD) is an acute febrile vasculitis primarily affecting young children.
  • The co-occurrence of MAS in KD presents diagnostic and therapeutic challenges.

Purpose of the Study:

  • To characterize the clinical features of KD patients presenting with MAS.
  • To review the treatment strategies and outcomes for these patients.
  • To emphasize the importance of early recognition and intervention for MAS in KD.

Main Methods:

  • Retrospective review of medical histories of KD patients diagnosed with MAS between January 2001 and March 2008.
  • Analysis of clinical signs, laboratory findings, and diagnostic criteria for MAS.
  • Evaluation of treatment protocols and patient outcomes.

Main Results:

  • 1.9% of 638 KD patients exhibited MAS features.
  • MAS was more common in males older than 5 years (OR: 6.8, P=0.002).
  • Key findings included prolonged fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, elevated D-dimers, hyperferritinemia, and elevated liver enzymes. Hemophagocytosis was confirmed in 4 patients. Most met MAS diagnostic criteria.
  • Treatment beyond standard KD care was required for most patients, leading to complete recovery without sequelae.

Conclusions:

  • KD patients with MAS require heightened clinical suspicion for timely diagnosis.
  • Aggressive treatment beyond standard KD protocols is often necessary for favorable outcomes.
  • Early detection and management of MAS in KD can prevent severe complications and ensure full recovery.

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