Clinical Practice Guidelines

Kawasaki Disease

  • Notes

    Kawasaki disease is a systemic vasculitis that predominantly affects children < 5 years of age. 

    Although the specific aetiological agent remains unknown, it is believed that Kawasaki disease is a response to some form of infection (though it is not transmitted from person to person).

    Diagnosis is often delayed because the features are similar to those of many viral exanthems. 

    The diagnostic criteria for Kawasaki disease are:

    Fever for 5 days or more, plus

    4 out of 5 of:

    • polymorphous rash
    • bilateral (non purulent) conjunctival injection
    • mucous membrane changes, e.g. reddened or dry cracked lips, strawberry tongue, diffuse redness of oral or pharyngeal mucosa
    • peripheral changes, e.g. erythema of the palms or soles, oedema of the hands or feet, and in convalescencedesquamation
    • cervical lymphadenopathy (> 15 mm diameter, usually unilateral, single, non purulent and painful)

    and exclusion of diseases with a similar presentation: staphylococcal infection (e.g. scalded skin syndrome, toxic shock syndrome), streptococcal infection (e.g. scarlet fever, toxic shock-like syndrome not just isolation from throat), measles, other viral exanthems,  Steven's Johnson syndrome, drug reaction and juvenile rheumatoid arthritis.

    Kawasaki disease pics

    Click to view photos & description

    The diagnostic features of Kawasaki disease can occur sequentially and may not all be present at the same time. Moreover, it is recognised that some patients with Kawasaki disease do not develop sufficient features to fulfill the formal diagnostic criteria. In particular, infants less than 12 months of age are both more likely to have incomplete criteria and more likely to develop coronary aneurysms.  Therefore, clinical vigilance is necessary to recognise these incomplete cases. This is important because the atypical cases are probably at similar risk of coronary complications and require treatment ( American Heart Association Algorithm Fr incomplete Kawasaki Disease.) Other relatively common features include arthritis, diarrhoea and vomiting, coryza and cough, uveitis, gall bladder hydrops. Some patients get myocarditis.

    Investigations

    All patients should have

    • ASOT / Anti DNAase B
    • Echocardiography (at least twice: at initial presentation and, if negative, again at 6 - 8 weeks).
    • Platelet count (marked thrombocytosis common in second week of illness)

    Other tests are not diagnostic or particularly useful. The following may be seen:

    • neutrophilia
    • raised ESR + CRP
    • mild normochromic, normocytic anaemia
    • hypoalbuminaemia
    • Elevated liver enzymes

    Thrombocytosis and desquamation appear in the second week of the illness or later. Their absence earlier does not preclude the diagnosis.

    Management

    Patients require admission to hospital if Kawasaki Disease is diagnosed or strongly suspected.

    • Intravenous immunoglobulin (2 g/kg over 10 hours; preferably within the first 10 days of the illness but should also be given to patients diagnosed after 10 days of illness if there is evidence of ongoing inflammation - eg fever, raised ESR/CRP)
      Administration of Intragam Guideline
    • Aspirin 3 - 5 mg/kg once a day for at least 6 to 8 weeks. 
      Some give a higher dose (10mg/kg 8 hourly for the first few days) but this probably adds nothing over immunoglobulin.

    Provide the Kawasaki Disease Parent Information Sheet.

    Parent Information Sheet  (Print version - PDF)

    Parent Information Sheet  (HTML version)

    Follow up

    Paediatric follow-up should be arranged on discharge. At least one further echocardiogram should be performed at 6-8 weeks. If this is normal, no further examinations are needed.