Diagnostic Guidelines of Kawasaki Disease

(MCLS : Infantile Acute Febrile Mucocutaneous Lymph Node Syndrome)

(The 5th Revised Edition, February 2002)

From Home Page of

Japan Kawasaki Disease Research Center (The authorized Non Profit Organization)

This is a disease of unknown etiology affecting most frequently infants and young children under 5 years of age. The symptoms can be classified into two categories, principal symptoms and other significant symptoms or findings.

A . PRINCIPAL SYMPTOMS

1. Fever persisting 5 days or more (inclusive of those cases in whom the fever has subsided before the 5th day in response to therapy)

2. Bilateral conjunctival congestion

3. Changes of lips and oral cavity: Reddening of lips, Strawberry tongue, Diffuse injection of oral and pharyngeal mucosa

4. Polymorphous exanthema

5. Changes of peripheral extremities:

Reddening of palms and soles, Indurative edema

Membranous desquamation from fingertips

6. Acute nonpurulent cervical lymphadenopathy

At least five items of 1-6 should be satisfied for diagnosis of Kawasaki disease. However, patients with four items of the principal symptoms can be diagnosed as Kawasaki disease when coronary aneurysm or dilatation is recognized by two–dimensional echocardiography or coronary angiography.

B. OTHER SIGNIFICANT SYMPTOMS OR FINDINGS

The following symptoms and findings should be considered in the clinical evaluation of suspected patients.

1. Cardiovascular : Auscultation (heart murmur, gallop rhythm, distant heart sounds), ECG changes (prolonged PR/QT intervals, abnormal Q wave, low-voltage QRS complexes, ST-T changes, arrhythmias), Chest X-ray findings (cardiomegaly), 2-D echo findings (pericardial effusion, coronary aneurysms), Aneurysm of peripheral arteries other than coronary (axillary etc.), Angina pectoris or Myocardial infarction

2. GI tract : Diarrhea, Vomiting, Abdominal pain, Hydrops of gall bladder, Paralytic ileus, Mild jaundice, Slight increase of serum transaminase

3. Blood : Leukocytosis with shift to the left, Thrombocytosis, Increased ESR, Positive CRP, Hypoalbuminemia, Increased α2-globulin, Slight decrease in erythrocyte and hemoglobin levels

4. Urine : Proteinuria, Increase of leukocytes in urine sediment

5. Skin : Redness and crust at the site of BCG inoculation, Small pustules, Transverse furrows of the finger nails

6. Respiratory : Cough, Rhinorrhea, Abnormal shadow on chest X-ray

7. Joint : Pain, Swelling

8. Neurological : CSF pleocytosis, Convulsion, Unconsciousness, Facial palsy, Paralysis of the extremities

REMARKS :

1. For item 5 under principal symptoms, the convalescent stage is considered important.

2. Non-purulent cervical lymphadenopathy is less frequently encountered (approximately 65%) than other principal symptoms during the acute phase.

3. Male : Female ratio : 1.3-1.5 : 1, patients under 5 years of age : 80-85%, fatality rate : 0.1%

4. Recurrence rate : 2-3%, proportion of siblings cases : 1-2%

5. Approximately 10 percent of the total cases do not fulfill five of the six principal symptoms, in which other diseases can be excluded and Kawasaki disease is suspected. In some of these patients coronary artery aneurysms (including so-called coronary artery ectasia) have been confirmed.

The Japan Kawasaki Disease Research Committee, c/o Department of Pediatrics, Japan Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-ku, Tokyo 150-8935, Japan

Phone : +81-3-3400-1311, fax : +81-3-3400-1394

The Committee deeply appreciates Professor Masato Takahashi of Childrens Hospital Los Angeles for his advice in translation of these Guidelines into English