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
- coronary aneurysms
- highest risk of death
- clinical symptoms and signs recede
- persistent of any cardiac complications into adulthood
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).
History and etymology
It is named after the Japanese physician (paediatrician) Tomisaku Kawasaki who initially described it in 1967 7.
- 1. Chung CJ, Stein L. Kawasaki disease: a review. Radiology. 1998;208 (1): 25-33. Radiology (citation) - Pubmed citation
- 2. Frey EE, Matherne GP, Mahoney LT et-al. Coronary artery aneurysms due to Kawasaki disease: diagnosis with ultrafast CT. Radiology. 1988;167 (3): 725-6. Radiology (abstract) - Pubmed citation
- 3. Bisset GS, Strife JL, Mccloskey J. MR imaging of coronary artery aneurysms in a child with Kawasaki disease. AJR Am J Roentgenol. 1989;152 (4): 805-7. AJR Am J Roentgenol (citation) - Pubmed citation
- 5. Duerinckx AJ, Troutman B, Allada V et-al. Coronary MR angiography in Kawasaki disease. AJR Am J Roentgenol. 1997;168 (1): 114-6. AJR Am J Roentgenol (citation) - Pubmed citation
- 6. Takemura A, Suzuki A, Inaba R et-al. Utility of coronary MR angiography in children with Kawasaki disease. AJR Am J Roentgenol. 2007;188 (6): W534-9. doi:10.2214/AJR.05.1414 - Pubmed citation
- 7. Kawasaki T. Mucocutaneous lymph node syndrome-clinical observation of 50 cases. Jpn J Allergy 1967;16: 178-182
- 8. Momolli MK, Castro E Silva Pretto JL, Sato D et-al. Calcified aneurysms in coronary arteries of a 48-year-old patient. Arq. Bras. Cardiol. 2001;76 (3): 255-60. Pubmed citation