Red eyes, Rash and Rising Ferritin: When Macrophages Make Mischief

Z Karaan

Background:
The detection of a respiratory virus is frequently assumed to explain persistent fever in young children, yet this assumption may dangerously delay recognition of Kawasaki disease (KD) and its most severe complications. Macrophage activation syndrome (MAS), though rare in KD, carries substantial mortality and is easily mistaken for severe infection.

Case:
A previously healthy 22-month-old boy presented with five days of fever, conjunctival injection, cough, and diarrhoea. He was irritable, tachypnoeic, and febrile, with bilateral crepitations and radiographic evidence of multifocal consolidation. Inflammatory markers were elevated, cultures were sterile, and nasopharyngeal PCR was positive for adenovirus. A diagnosis of adenoviral pneumonia was made and supportive care with empiric antibiotics initiated.
Despite appropriate management, fever persisted and clinical features evolved over the subsequent six days, including cervical lymphadenopathy, oral mucositis, and a desquamating perineal rash. Laboratory trends revealed escalating hyperinflammation with bicytopenia, transaminitis, hyperferritinaemia, and hypertriglyceridaemia, raising concern for MAS. Echocardiography demonstrated coronary artery dilatation. KD complicated by MAS and coronary arteritis was diagnosed, despite the presence of a confirmed viral infection.
The child was treated with intravenous immunoglobulin, high-dose methylprednisolone, and aspirin, resulting in rapid defervescence, biochemical recovery, and rebound thrombocytosis. Coronary abnormalities resolved completely by six-week follow-up.

Conclusion:
This case underscores a critical diagnostic pitfall: a positive viral PCR does not exclude KD or its life-threatening complications. MAS should be actively considered in children with KD who demonstrate disproportionate hyperinflammation or evolving cytopenias. Serial clinical examination and trend-based interpretation of laboratory data – not single time-point results – are essential. Early recognition and prompt immunomodulatory therapy can be lifesaving and coronary-sparing, even when KD presents behind the mask of acute viral infection.