Kawasaki Disease

Practice Gap

1. Clinicians should not dismiss the diagnosis of Kawasaki disease (KD) in children with symptoms commonly attributed to viral illness. For example, severe headache and photophobia should signal the possibility of aseptic meningitis even in the presence of KD. And, right upper quadrant pain may indicate hydrops of the gallbladder.

2. A challenging subset of patients who do not meet the classic case definition are said to have incomplete KD. Patients who have incomplete KD are more likely to be infants and older children and, as such, are also at higher risk for coronary artery lesions (CAL). Of note, infants younger than 6 months of age are at high risk for development of CAL, yet often have fewer clinical features to facilitate the diagnosis. For these reasons, echocardiography is recommended for infants younger than age 6 months with fever of unclear etiology persisting for 7 or more days and elevated inflammatory markers.

Objectives

After reading this article, readers should be able to:

1. Describe the clinical manifestations of Kawasaki disease.

2. Formulate a differential diagnosis for patients with suspected Kawasaki disease.

3. Describe the laboratory values typically seen in Kawasaki disease.

4. Discuss the role of echocardiography in the management of patients who have Kawasaki disease and describe the cardiac complications of the disease.

5. Define primary treatment of Kawasaki disease with intravenous immunoglobulin and aspirin.

Case Study

A 3-year-old previously healthy Hispanic girl is brought to her pediatrician’s office with a history of 6 days of fever. The fever has been present daily and has been unremitting, despite administration of antipyretic medications. She has been irritable with decreased appetite. Her mother noticed an erythematous, nonpruritic rash covering her torso 1 day after …