Kawasaki disease

Disclaimer

These guidelines have been produced to guide clinical decision making for the medical, nursing and allied health staff of Perth Children’s Hospital. They are not strict protocols, and they do not replace the judgement of a senior clinician. Clinical common-sense should be applied at all times. These clinical guidelines should never be relied on as a substitute for proper assessment with respect to the particular circumstances of each case and the needs of each patient. Clinicians should also consider the local skill level available and their local area policies before following any guideline. 

Read the full PCH Emergency Department disclaimer.

Aim

To guide staff with the assessment and management of Kawasaki Disease.

Background

  • Kawasaki disease is a common vasculitis of childhood especially in < 5 year olds
  • Aetiology is unknown
  • Is a self-limiting condition, with fever and manifestations of acute inflammation lasting an average 12 days without therapy, however, early treatment is necessary to prevent complications.

Complications

  • Coronary artery aneurysms
    • Infants under 12 months are at increased risk of coronary artery aneurysm
    • Delay of treatment (after 10 days) increases risk of coronary artery aneurysm by five times
  • Depressed myocardial contractility and heart failure
  • Myocardial infarction
  • Arrhythmias
  • Peripheral arterial occlusion.

Assessment

Diagnostic criteria

Kawasaki Disease is a clinical diagnosis with no diagnostic laboratory test.

Presence of prolonged unexplained fever ≥ 5 days (fever > 38.5°C) with at least 4 of the following criteria:

  1. Bilateral non-exudative conjunctivitis
  2. Polymorphous rash
  3. Cervical lymphadenopathy (at least 1 lymph node >1.5cm in diameter)
  4. Mucositis - cracked red lips, injected pharynx or strawberry tongue
  5. Extremity changes - erythema of palms/soles, oedema of hands/feet (acute phase), and periungual desquamation (convalescent phase)

Associated non-specific symptoms

  • Diarrhoea, vomiting, or abdominal pain
  • Irritability
  • Cough or rhinorrhea
  • Joint pain
  • Weakness.

Incomplete (atypical) Kawasaki Disease

  • Diagnostic criteria not completely fulfilled (< 4 signs of mucocutaneous inflammation) but otherwise similar clinical picture to that of 'classic' Kawasaki disease
  • More likely with children < 12 months old and > 5 years old
  • Atypical Kawasaki Disease patients are still at risk of cardiovascular complications
    • If prolonged unexplained fever discuss with ED consultant/on call general paediatric consultant.

Laboratory findings (not diagnostic but supportive)

  • Elevated acute phase reactants (CRP, ESR)
  • Elevated WCC with predominant neutrophilia
  • Elevated platelets (after 1 week)
  • Normocyctic, normochromic anaemia
  • Sterile pyuria (need clean voided specimen).

Investigations

No diagnostic lab tests for Kawasaki Disease but can be supportive or used to exclude other causes of fever.

  • CPR, ESR, FBC, ALT, Albumin
  • ASOT/AntiDNAse B
  • Urinalysis - clean catch or in-out catheter
  • Blood culture.

Differential diagnoses

  • Adenovirus
  • Epstein Barr Virus
  • Scarlet fever
  • Toxic Shock Syndrome
  • Steven-Johnson Syndrome
  • Measles.

Management

All suspected cases should be discussed with the ED admitting registrar/consultant for admission under the on call general paediatric consultant.

Initial management

  • IV Immunoglobulin (IVIG) 2 grams/kg over 8-12 hours
  • Low dose aspirin at 3-5mg/kg daily.

Further management

  • Second dose IVIG may be given if incomplete treatment response, particularly in high risk age group and 'atypical' Kawasaki disease
  • General paediatric team will refer the patient to the cardiology team for echocardiogram only after the diagnosis of Kawasaki Disease is made/confirmed and treatment instituted
  • Many patients (especially if < 3 years old) will require sedation to perform the echocardiogram as the irritability (commonly seen) precludes performing adequate echocardiogram in the acute phase
  • Echocardiogram is required at/after initial diagnosis and repeated at 4-8 weeks post treatment
  • Echocardiogram plays no role in the diagnosis of Kawasaki Disease.

Discharge treatment

  • Continue aspirin (low dose) at 3-5mg/kg daily until the repeat echocardiogram at 4-8 weeks confirms absence of coronary involvement.

Bibliography

  1. Sundal R (2014) Kawasaki Disease: Clinical Features and Diagnosis. UpToDate. Accessed at www.uptodate.com
  2. Sundal R (2014) Kawasaki Disease: Initial Treatment and Prognosis. UpToDate. Accessed at www.uptodate.com 
  3. MH Children's Dosing Companion (online). Adelaide: Australian Medicines Handbook Pty Ltd; 2015 January. Available from: https://childrens-amh-net-au.pklibresources.health.wa.gov.au 
  4.  AHA SCIENTIFIC STATEMENT :Diagnosis, Treatment, and Long-Term Management of Kawasaki DiseaseA Scientific Statement for Health Professionals From the American Heart Association Brian W. McCrindle, MD,MPH, FAHA, ChairAnne H. Rowley, MDJane W. Newburger, MD.


Endorsed by:  Director, Emergency Department   Date:  Mar 2018


 Review date:   Mar 2021


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