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(Circulation. 2001;103:335.)
© 2001 American Heart Association, Inc.

AHA Scientific Statement

Diagnostic Guidelines for Kawasaki Disease

Council on Cardiovascular Disease in the Young Committee on Rheumatic Fever Endocarditis and Kawasaki Disease American Heart Association

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 -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:

1. Changes in extremities: Acute: Erythema and edema of hands and feet Convalescent: Membranous desquamation of fingertips
   
2. Polymorphous exanthema
   
3. Bilateral, painless bulbar conjunctival injection without exudate
   
4. Changes in lips and oral cavity: Erythema and cracking of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosae
   
5. 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

Selected Readings

Newburger JW, Takahashi M, Beiser AS, et al. A single intravenous infusion of gamma globulin compared with four infusions in the treatment of acute Kawasaki syndrome. N Engl J Med. 1991;324:1633–1639.

Dajani AS, Taubert KA, Gerber MA, et al. Diagnosis and management of Kawasaki disease in children. Circulation. 1993;87:1776–1780.

Taubert KA, Rowley AH, Shulman ST. Seven-year national survey of Kawasaki disease and acute rheumatic fever. Pediatr Infect Dis J. 1994;13:704–708.

Dajani AS, Taubert KA, Takahashi M, et al. Guidelines for long-term management of patients with Kawasaki disease: report from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation. 1994;89:916–922.

Shulman ST, Rowley AH. Kawasaki syndrome. In: Jensen HB, Baltimore RS, eds. Pediatric Infectious Diseases: Principles and Practice. Norwalk, Conn: Appleton & Lange; 1995:629–638.

Kato H, ed. Kawasaki Disease: Proceedings of the 5th International Kawasaki Disease Symposium, Fukuoka, Japan. Amsterdam: Elsevier; 1995.

Yanagawa H, Nakamura Y, Yashiro M, et al. Update of the epidemiology of Kawasaki disease in Japan: from the results of 1993–94 nationwide survey. J Epidemiol. 1996;6:148–157.

Kato H, Sugimura T, Akagi T, et al. Long-term consequences of Kawasaki disease: a 10- to 21-year follow-up study of 594 patients. Circulation. 1996;94:1379–1385.

Shulman ST, Rowley AH. Etiology and pathogenesis of Kawasaki disease. Prog Pediatr Cardiol. 1997;6:187–192.

Taubert KA. Epidemiology of Kawasaki disease in the United States and worldwide. Prog Pediatr Cardiol. 1997;6:181–185.

Taubert KA, Shulman ST. Kawasaki disease. Am Fam Phys. 1999;57:3093–3102.

Barron KS, Shulman ST, Rowley A, et al. Report of the National Institutes of Health workshop on Kawasaki disease. J Rheumatol. 1999;26:170–190.

View larger version (120K): [in this window] [in a new window]   Figure 1. Rash of Kawasaki disease in a 7-month-old on the 4th day of illness.

View larger version (124K): [in this window] [in a new window]   Figure 2. Conjunctival injection, lip edema, and erythema in a 2-year-old boy on the 6th day of illness.

View larger version (118K): [in this window] [in a new window]   Figure 3. Erythematous and edematous hand of a 11/2-year-old girl on the 6th day of illness.

View larger version (116K): [in this window] [in a new window]   Figure 4. Periungual desquamation in a 3-year-old on the 12th day of illness.

View larger version (132K): [in this window] [in a new window]   Figure 5. Two-dimensional echocardiogram. AO indicates aorta; PA, pulmonary artery; LAD, left anterior descending coronary; CX, circumflex coronary; and L Main, left main coronary.

View larger version (141K): [in this window] [in a new window]   Figure 6. Coronary angiogram demonstrating hugely dilated left anterior descending (LAD) artery with obstruction and very dilated right coronary artery (RCA) with an area of severe narrowing in a 6-year-old boy.

Footnotes

For reprint requests, contact ktaubert@heart.org.

Adapted in part from the Japan Kawasaki Disease Research Committee.

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