Clinical Practice Guidelines

Kawasaki disease


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    This guideline has been adapted for statewide use with the support of the Victorian Paediatric Clinical Network

  • See also:

    Key Points

    1. Kawasaki Disease (KD) is a clinical diagnosis that requires prompt recognition and management.
    2. Early treatment with intravenous immunoglobulin (IVIg) has been shown to reduce morbidity and mortality.

    Background

    KD is the second most common vasculitis in childhood after Henoch Schonlein purpura, and is the most commoncause of acquired heart disease in children in developed countries causing coronary artery aneurysms (CAA).

    It has a worldwide distribution, although is more common in Asian children.

    Approximately 85% of cases occur under 5 years of age, peak age 18-24 months

    • KD in children < 6 months and >5 years is less common, however these children are more likely to develop CAA.

    The disease often presents without all diagnostic criteria (see below) and can present a significant diagnostic challenge to the clinician.

    Assessment

    Kawasaki Disease: Diagnostic criteria. 

    Fever persisting for at least 5 days, PLUS 4 of the 5 criteria:

    NB.  KD can be diagnosed with less than four of the following features if coronary artery abnormalities are present.

    Criterion                                                   Features

    Conjunctivitis
    Conjunctivitis 1
    Conjunctivitis 2

    Bilateral, "dry" or non-purulent, painless.  Preferentially bulbar in distribution. 

    Lymphadenopathy

    Lymphadenopathy  

    Cervical, most commonly unilateral, tender.  At least one node >1.5cm.

    Rash

    Rash 1

    Rash 2

    Polymorphous; without vesicles, bullae or crusts; occurring in the first few days, involves the trunk and extremities. 

    Variable presentations such as urticarial, morbilliform, maculopapular, or resembling scarlet fever.

    Lips and oral mucosa

    Lips and oral mucosa 1

    Lips and oral mucosa 2

    Intense hyperaemia of lips leading to redness and cracking and/or diffuse erythema of oropharynx.  Strawberry tongue.

    Extremities

     Extremities 1

    Extremities 2

    Hyperaemia and painful oedema of hands and feet that progresses to desquamation in the convalescent stage.

    Perineal desquamation frequently associated.

       Photographs used with permission from the Kawasaki Disease Foundation, Inc

    Irritability is an important sign and one that is very frequently present, although not included as a diagnostic criterion. 

    Diagnostic features may present sequentially.  KD should be considered in any child with fever, rash and evidence of systemic inflammation.

    Common findings outside the diagnostic criteria include arthritis, aseptic meningitis, sterile pyuria and dysuria.

    Pitfalls

    It is important to recognise the potential for missed diagnosis in infants:

    • < 6 months with prolonged fever and irritability without other features.
    • with prolonged fever and unexplained aseptic meningitis.
    • with prolonged fever and cervical adenitis not responsive to oral antibiotics.
    Differential diagnosis
    • Group A Streptococcal infections – tonsillitis, Scarlet fever, acute rheumatic fever
    • EBV, Adenovirus
    • Systemic juvenile idiopathic arthritis (JIA)
    • Sepsis or Toxic Shock syndrome
    • Stevens-Johnson Syndrome
    • Drug reaction

    Management

    Investigations

    • Echocardiogram – at baseline (this should not delay initiation of treatment) and at 6 weeks. Abnormalities should be managed in consultation with paediatric cardiology and haematology services.
    • In all patients consider:
      • FBE, CRP, ESR, UEC, LFT (NB ESR is unreliable after IVIg administration)
      • Blood culture
      • Serum to store
      • Urinalysis (sterile pyuria)
      • ECG

    Although nonspecific, laboratory tests provide support for diagnosis, assessment of severity and monitoring of disease and treatment.

    Common abnormalities include elevation of ESR, CRP and WCC. Thrombocytosis is common in the second week of illness.

    Incomplete Kawasaki Disease:

    • Consideration of incomplete KD can present a significant diagnostic dilemma.
    • The American Heart Association has proposed the following algorithm for the evaluation of suspected Incomplete Kawasaki Disease:
      Kawasaki disease diagram     

    Treatment

    1. INTRAVENOUS IMMUNOGLOBULIN (IVIg): 2g/kg as a single IV infusion on diagnosis.
      • IVIg should always be given 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.
      • A second dose of 2g/kg IVIg should be given to patients who do not respond to the first dose, as demonstrated by persistent or recurrent fevers after 36 hours.  This should be done in close consultation with a paediatrician.
      • The National Blood Authority and BloodSTAR coordinate and authorise the use of blood products.  IVIg is a product that must be ordered via their website.
      • Local hospital blood banks should be consulted regarding processes required.
    2. CORTICOSTEROIDS: The use of corticosteroids in the treatment of KD remains controversial. Consider for high risk patients in discussion with local paediatric team. More likely to be beneficial at the commencement of treatment for KD in high risk patients, rather than after a failure of initial IVIg treatment.  High risk as suggested by:
      • Signs of shock.
      • Patients < 12 months of age.
      • Asian ethnicity.
      • ALT > 100 IU/L
      • Albumin < 30 g/L
      • Any patient with evidence of cardiac involvement on echocardiography at time of presentation.
    3. Prednisolone 2mg/kg (max 60mg) orally daily for a minimum of 5 days and until CRP normalises.  (Evidence for optimal dose/duration is limited)

      A steroid course of >10 days will require weaning and consideration of concurrent proton pump inhibitor or H2 receptor blocker.

    4. ASPIRIN:
      3-5mg/kg as a daily dose until normal echo on follow up (minimum 6 weeks).
      The association of Reye syndrome with aspirin remains a consideration, thus risks must be balanced against clinical benefit.

    Consider consultation with local paediatric team when:

    ALL children with diagnosed or suspected Kawasaki Disease should be discussed with the local paediatric unit and admitted.

    Consider transfer when:

    • Child has cardiac involvement (in consultation with the paediatric cardiology team).
    • Child does not respond to initial treatment.
    • Children requiring care above the level of comfort of the local hospital.

    For emergency advice and paediatric or neonatal ICU transfers, call the Paediatric Infant Perinatal Emergency Retrieval (PIPER) Service: 1300 137 650.

    Consider discharge when:

    • Afebrile and well at least 36 hours after treatment.
    • Children are discharged on a daily dose of aspirin (see above).
    • Follow up should include repeat echocardiogram at 6 weeks after initial treatment and general paediatric review.

    Parent information sheet: Kawasaki Disease

    Information specific to RCH: Early referral to Cardiology for consideration of echocardiography is advised.

    Last updated August, 2017