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Kawasaki Disease (KD)

NICE guideline [NG143] Fever in under 5s: assessment and initial management. Kawasaki Disease. Last updated: Nov 2021. Update on Diagnosis and Management of Kawasaki Disease: A Scientific Statement From the American Heart Association

Diagnosis Guidelines

KD is diagnosed clinically, there are no specific diagnostic tests.

Clinical features:
  • Fever ≥ 5 days
  • Principal features (CRASH)
    • Conjunctival infection (bilateral without exudate)
    • Rash (polymorphous)
    • Adenopathy (cervical - usually unilateral)
    • Strawberry tongue and other oral changes (lips erythema and cracking, oral and pharyngeal mucosa erythema)
    • Hands and feet erythema and swelling

 

NICE recommends considering KD in ALL children with fever lasting ≥ 5 days.

  • NICE noted that it is common that not all the principal features are present in KD
  • Note that the AHA diagnostic criteria require 4 out of 5 of the principal features to be present

Most important test: echocardiography
  • Purpose: to check for coronary artery aneurysm (most frequent location: proximal left anterior descending artery and proximal right coronary artery)
  • Note that normal echocardiography does NOT exclude diagnosis, therefore should NOT delay initiation of treatment

Management Guidelines

Standard 1st line:
  • IVIG single dose, and
  • Low-dose aspirin for at least 6 weeks

 

IVIG is the most important treatment in KD, to rapidly reduce inflammation and lower the risk of coronary artery aneurysm development. It should be given within 10 days of fever onset.

Aspirin's role is primarily for cardiovascular protection via its antiplatelet effect.

A well-established risk of aspirin use in children is the development of Reye's syndrome. Therefore, aspirin is contraindicated in children, with the exception of its use in KD.

Despite the risk of Reye's syndrome with aspirin in children, it remains a standard treatment for KD. As the benefits of aspirin in KD outweigh the risks due to the significant risk of coronary artery damage in KD.

Further therapy (for refractory disease / IVIG-resistant cases / coronary artery aneurysm - under specialist care):
  • Corticosteroids
  • Immunomodulators
    • Anti-TNF alpha (infliximab, etanercept)
    • Ciclosporin
    • Anakinra
    • Cyclophosphamide

References

Author: Adams Lau
Reviewer:
Last edited: 26/08/25