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Kawasaki disease (KD), a systemic vasculitis, is the leading cause of acquired heart disease in industrialised countries. Despite decades of research, the aetiology and pathogenesis remain unclear. Prompt diagnosis and management of KD are essential to reduce the risk of coronary artery damage that may cause significant morbidity, including risk of myocardial ischaemia or infarction, and occasional mortality. In practice, this is often difficult due to a lack of a diagnostic test and relies on the recognition of a constellation of clinical features.
Since the American Heart Association (AHA) guideline in 2004,1 there have been over 2000 scientific and clinical publications on KD. The recently published 2017 AHA guideline, a scientific statement, provides detailed updated recommendations on the diagnosis, management and treatment of KD.2 Here we highlight and summarise the key updates of particular relevance to paediatricians and discuss other recent advances.
Key points from the 2017 AHA Kawasaki disease guideline
Diagnosis of KD requires recognition of a constellation of clinical features (box 1).
KD can now be diagnosed with 4 days of fever when at least four principal diagnostic criteria are present (particularly if one of these is erythema and oedema of the hands and feet).
KD should still be considered if the fever resolves after 7 days without treatment.
KD should also be considered in:
Infants under 6 months of age with prolonged fever and irritability.
Infants with prolonged fever and aseptic meningitis.
Infants or children with prolonged fever plus unexplained lymphadenitis, retrotonsillar collection or shock.
Diagnostic clinical criteria for classical Kawasaki disease
Presence of fever for at least 5* days with at least four of the five following clinical features:
Erythema and cracking of lips, strawberry tongue and/or erythema of oral and pharyngeal mucosa.
Bilateral bulbar conjunctival injection without exudate.
Rash: maculopapular, diffuse erythroderma, or erythema multiforme-like.
Erythema and oedema of the hands and feet in acute phase and/or periungual desquamation …
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