Kawasaki Disease is a multisystem inflammatory disease that affects mostly infants and children. It has replaced rheumatic fever as the most common cause of acquired heart disease in North America, Japan, and Europe

Epidemiology

1. Incidence is 2/100,000/yr in African Americans and Caucasians and 5/100,000/yr. in Asians

2.Peak age is 18-24 months and 80-90% are less than 5 years old. M:F=1.5:1

Etiology

1. Unknown although infectious agent highly suspicious because of seasonal peak in summer and winter and self limiting course

Diagnosis

1. The presence of unexplained fever for 5 days or more and the presence of 4 of the following 5 findings;

a. Bilateral bulbar conjunctival injection without discharge and sparing of the limbus

b. Mucous membrane changes- dryness and fissuring of the lips, erythema of the oral mucosa, strawberry tongue.

c. Polymorphous generalized rash- never vesicular or bullous, may have perineal desquamation

d. Peripheral extremity changes- erythema of the palms and soles, periungal desquamation of fingers and toes

e. Nonsuppurative lymphadenopathy- > 1.5 cm

Other Clinical Findings

1. Irritability

2. Abdominal pain and pseudobstruction

3. Diarrhea and vomiting

4. Mild hepatic dysfunction

5. Urethritis with sterile pyuria

6.Arthralgias and arthritis

7. Aseptic meningitis

8. Hydrops of the Gallbladder

9. Coronary Aneurysms- develop in ~20% of untreated patients and < 5% of treated patients. Higher incidence in < 1 year of age, males, and when here has been prolonged fever and inflammation.

10. CHF, myocarditis, pericarditis, mitral insufficiency

Laboratory findings

1. Elevated acute phase reactants -CRP, ESR, Thrombocytosis, leukocytosis

2. Normochromic normocytic anemia

3. Sterile pyuria

4. Mild elevation of liver enzymes and bilirubin

5. Mononuclear cells in the CSF

Diagnostic Work-up

1. When there is clinical suspicion of Kawasaki Disease, an Echocardiogram should be performed.

2. Other etiologies of the clinical picture must be ruled out.-Measles, Scarlet Fever, Drug reactions, other viral illnesses, RMSF, Leptospirosis

3. Clinical Diagnosis

Management

1. IVIG- usually rapid response with child becoming afebrile and feeling better

2. High Dose Aspirin- continue until patient is afebrile and in patients without coronary aneurysms, low dose ASA until all markers of inflammation are normal

3. Failure to respond to IVIG may require another dose.

4. Follow-up with cardiologist. Persistence of coronary aneurysms may require periodic cardiac examinations and continuance of low dose ASA.

Prognosis

1. Coronary aneurysms demonstrate regression in 2 years

2. Giant aneurysms have higher risk of stenosis or complete obstruction

References

1. Eagle K. Kawasaki Disease NEJM Vol 333 Nov 23, 1995 pg 1391

2. Nakamura Y et al.. Mortality Among Persons with a History of Kawasaki Disease in Japan. Archives of Pediatrics and Adolescent Medicine. Feb. 2002

3. Waseem M. Pinkert H. Visual Diagnosis Pediatrics in Review 24: 245-248