Kawasaki disease

Kawasaki disease is a small to medium vessel vasculitis predominantly affecting young children. It can affect any body organ but there is a predilection for the coronary vessels.

An autoimmune aetiology has been postulated. It is generally self-limiting but acute fatalities are thought to occur in ~1% of cases. The most concerning morbidity is due to coronary involvement where it can manifest as a myocarditis with coronary arterial aneurysm formation 1,2,4.

Persisting fever is the most common way most children present to medical attention. The specific signs and symptoms vary with the stage of disease.

  • fever that doesn't respond to antibiotics
  • general malaise and irritability
  • non-exudative conjunctivitis (90%)
  • anterior uveitis (70%)
  • perianal erythema (70%)
  • lymphadenitis (75%)
  • strawberry tongue
  • desquamation of the digits
  • thrombocytosis
  • coronary aneurysms
  • highest risk of death
  • clinical symptoms and signs recede
  • persistent of any cardiac complications into adulthood

Chest radiographs can be normal. Abnormal findings are non-specific and include a reticulogranular pattern, peribronchial cuffing, a pleural effusion, atelectasis and/or air trapping 1.

Rarely, a few years after resolution of the initial episode, the patient may present with calcified coronary artery aneurysms visible on the chest x-ray 8. Although rare, this is an Aunt Minnie presentation of Kawasaki disease sequelae in older patients.

May show small coronary arterial ectasiae, aneurysms or stenoses. Angiography is the most sensitive and specific for vascular assessment 4.

Useful in assessing myocardial perfusion, wall thinning as well as aneurysms (MRA).

It is named after the Japanese physician (paediatrician) Tomisaku Kawasaki who initially described it in 1967 7.

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Article information

rID: 7431
Synonyms or Alternate Spellings:
  • Kawasaki disease
  • Mucocutaneous lymph node syndrome
  • Kawasaki disease (KD)
  • Kawasaki's disease

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Cases and figures

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    Case 1: with RCA and LAD aneurysms
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