Issues in the diagnosis of Kawasaki disease

The diagnosis of Kawasaki disease (KD) is based on clinical features and supporting laboratory and echocardiographic findings. Diagnosis is often difficult as the etiology remains unknown and no specific diagnostic assay exists, and many of the clinical features mimic common childhood viral illnesses. Incomplete (or atypical) KD is being recognized more frequently, and diagnosis may be difficult because fewer clinical features are present; however, diagnosis remains essential as these patients develop coronary artery sequelae at least as frequently as children with classic KD. Infants are at particular risk for coronary artery sequelae and often have incomplete KD features requiring astute clinical acumen. Revised guidelines for the diagnosis of incomplete KD are being developed, but until a diagnostic assay is available, a low threshold for theraphy is required to avoid potentially devastating sequelae.