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TABLE 101.15
Pathology of Kawasaki Disease a
Stage I—Disease duration <10 days
Acute perivasculitis of coronary arteries
Microvascular angiitis of coronary arteries and aorta
Pancarditis with pericardial, myocardial, endocardial inflammation
Inflammation of the atrioventricular conduction system
Stage II—Disease duration 12–28 days
Acute panvasculitis of coronary arteries
Coronary artery aneurysms present
Coronary obstruction and thrombosis
Myocardial and endocardial inflammation less intense
Stage III—Disease duration 28–45 days
Subacute inflammation in coronary arteries
Coronary artery aneurysms present
Myocardial, endocardial inflammation much decreased
Stage IV—Disease duration >50 days
Scar formation, calcification in coronary arteries
Stenosis and recanalization of coronary vessel lumen
Myocardial fibrosis without acute inflammation
a Duration

of each stage may be decreased by prompt treatment with IVIG.
IVIG, intravenous immunoglobulin.

Goals of Treatment
Early recognition of the symptoms of KD is essential to effective and timely therapy.
Although no definitive test for KD exists, recognition of the constellation of signs and
symptoms is instrumental in making the diagnosis. The goals of treatment are to reduce
the amount of ectasia and aneurysms that occur among the coronary arteries which can
be accomplished by the initiation of IVIG therapy within the first 10 days of the illness.

Clinical Considerations
Clinical Recognition
KD is a clinical syndrome diagnosed on the basis of fever and four of the five signs of
mucocutaneous inflammation ( Table 101.16 ). These diagnostic criteria should be
regarded as imperfect, with <100% sensitivity and specificity. Children who do not
meet criteria may indeed have KD, whereas some children with other conditions may
nonetheless manifest all five criteria of KD. Acknowledgment of this fact was made