Kawasaki disease, or mucocutaneous lymph node syndrome, is a disease of unknown etiology that most frequently (80% of the time) affects infants and children under 5 years of age. Accurate diagnosis and early therapeutic interventions such as aspirin and intravenous γ-globulin can decrease the approximately 20% risk of developing coronary artery abnormalities. A specific diagnostic test does not exist. Thus, diagnosis of Kawasaki disease is based on characteristic clinical signs and symptoms, which are classified as principal clinical findings and other clinical and laboratory findings. The male-to-female ratio among patients with Kawasaki disease is 1.5:1. Children of nearly all racial backgrounds are affected. Recurrences and cases in siblings are seen only occasionally.
Principal Clinical Findings*
Fever persisting at least 5 days† and the presence of at least 4 of the following 5 principal features:
Changes in extremities: Acute: Erythema and edema of hands and feet Convalescent: Membranous desquamation of fingertips
Bilateral, painless bulbar conjunctival injection without exudate
Changes in lips and oral cavity: Erythema and cracking of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosae
Cervical lymphadenopathy (≥1.5 cm in diameter), usually unilateral
*Patients with fever and fewer than 4 principal symptoms can be diagnosed as having Kawasaki disease when coronary artery disease is detected by 2-dimensional echocardiography or coronary angiography. Other diagnoses should be excluded. The physician should be aware that some children with illness not fulfilling these criteria have developed coronary artery aneurysms.
†Many experts believe that in the presence of classic features, the diagnosis of Kawasaki disease can be made by experienced observers before day 5 of fever.
Other Significant Clinical and Laboratory Findings
Cardiovascular: On auscultation, gallop rhythm or distant heart sounds; ECG changes (arrhythmias, abnormal Q waves, prolonged PR and/or QT intervals, occasionally low voltage, or ST-T–wave changes); chest x-ray abnormalities (cardiomegaly); echocardiographic changes (pericardial effusion, coronary aneurysms, or decreased contractility); mitral and/or aortic valvular insufficiency; and rarely, aneurysms of peripheral arteries (eg, axillary), angina pectoris, or myocardial infarction
Gastrointestinal: Diarrhea, vomiting, abdominal pain, hydrops of gallbladder, paralytic ileus, mild jaundice, and mild increase of serum transaminase levels
Blood: Increased erythrocyte sedimentation rate, leukocytosis with left shift, positive C-reactive protein, hypoalbuminemia, and mild anemia in acute phase of illness (thrombocytosis in subacute phase)
Urine: Sterile pyuria of urethral origin and occasional proteinuria
Skin: Perineal rash and desquamation in subacute phase and transverse furrows of fingernails (Beau’s lines) during convalescence
Respiratory: Cough, rhinorrhea, and pulmonary infiltrate
Joint: Arthralgia and arthritis
Neurological: Mononuclear pleocytosis in cerebrospinal fluid, striking irritability, and rarely, facial palsy
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Adapted in part from the Japan Kawasaki Disease Research Committee.
- Copyright © 2001 by American Heart Association