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Kawasaki Syndrome

Kawasaki syndrome (KS) is an acute, febrile, self-limited, infectious, multisystem vasculitis, which occurs in young children. Fever is often prolonged and coronary aneurysms may lead to myocardial infarction and death.

Clinical Manifestations

Abrupt onset of high but remittent fever between 38 and 41 degrees C is characteristic.

Within 2 to 5 days, the child develops other diagnostic signs of KS: Conjunctival injection, mouth changes, an erythematous rash, changes in the hands and feet.

Eye involvement consists of conjunctival injection and, often, photophobia.

The lips are initially bright red, progressing over 3 days to swelling, cracking, and bleeding.

The skin rash is deeply erythematous with slightly raised margins, varying in size from 2 to 3 mm papules to large plaques covering several centimeters. The rash often is urticarial and may be intensely pruritic.

Firm, indurative edema of the hands and feet and diffuse red-purple discoloration of palms and soles.

Ten to 20 days after the onset of fever, in early convalescence, desquamation starts just under the nails.

Cervical lymph node involvement occurs in 50% of patients, manifesting as sudden onset.

Kawasaki Disease: Diagnostic Criteria

I. Fever for >5 days (usually >102EF)

II. At least four of five features

A. Bilateral conjunctival injection (bulbar. non-purulent)

B. Cervical adenitis (unilateral >1.5 cm diameter, non-fluctuant)

C. Rash (truncal. perineal accentuation, polymorphous but non-vesicular)

D. Inflamed oral mucosae (fissured lips, strawberry tongue)

E. Hands and feet inflammation (periungual peeling around 14-21 days)

III. No alternate diagnosis

IV. Fever plus 3/5 criteria are diagnostic when coronary abnormalities are present

    Associated Features

    Extreme irritability and emotional lability is common.

    Mild cerebrospinal fluid pleocytosis occurs in 25%. The CSF cell count is between 50 and 150/mm3 and is mononuclear. Protein levels are normal to slightly elevated, and glucose level is normal.

    Urethritis is present in 60% and is characterized by sterile pyuria. Red blood cells may be detected.

    Cardiac Manifestations

      Sixty percent have tachycardia with gallop rhythm, and 20% have congestive heart failure. Thirty percent have a pericardial effusion, and 30% have tricuspid insufficiency.

      Prolongation of the PR interval and first-degree heart block are very common, but more significant arrhythmias are rare. Eighteen to 25% of patients.

    Treatment of Kawasaki Syndrome

      Intravenous Gamma Globulin. As soon as KS is diagnosed, a baseline echocardiogram is obtained and IVIG 2 g/kg is given in an 8- to 12-hour infusion. Heart rate and blood pressure.

Aspirin

    High dose aspirin therapy is started on the same day as IVIG. The aspirin dosage is 100 mg/kg/day until a few days after defervescence or until the 14th day of illness.

    Serum salicylate levels should be obtained if symptoms of toxicity (vomiting, hyperpnea, lethargy) or liver function abnormalities develop.

Prognosis

KS usually is self-limited; however, cardiac damage may be serious. Twenty percent of all patients not treated with IVIG develop coronary artery aneurysms, appearing 7 days to 4 weeks after the onset.