Kawasaki disease

Kawasaki disease (KD) 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.

Acute stage
  • 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
Subacute stage
  • desquamation of the digits
  • thrombocytosis
  • coronary aneurysms
  • highest risk of death
Convalescent stage
  • clinical symptoms and signs recede
Chronic stage
  • persistent of any cardiac complications into adulthood
Plain radiograph

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.

Coronary angiography / CT angiography

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

MRI / MR angiography

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

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