Kawasaki Disease

Objectives

After completing this article, readers should be able to:

1. Describe the clinical and laboratory manifestations of Kawasaki disease (KD).

2. Describe the features of atypical, or incomplete, KD.

3. Discuss the value of high-dose aspirin and intravenous immune globulin (IVIG) in the treatment of KD and possible therapies for IVIG-refractory KD.

4. Identify the cardiac complications of KD and the importance of evaluation and follow-up.

5. Delineate the prognosis and management of KD.

Introduction

Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown cause that has a striking predilection for the coronary arteries of infants and young children. First described in Japan in 1967 by Tomisaku Kawasaki, the disease now is known to occur in the Americas, Europe, and Asia in children of all races. In retrospect, the entity known as infantile polyarteritis nodosa likely is a part of the continuum of KD, and clinically mild KD probably was confused with diseases such as measles or scarlet fever before the advent of vaccines and antibiotics. In fact, old reports on infantile polyarteritis nodosa describe pathologic findings that are identical to those of fatal KD.

Epidemiology

KD is markedly more prevalent in Japan and in children of Japanese ethnicity, having an annual incidence of 150 cases per 100,000 children younger than 5 years of age, about 10 times the rate in the United States. In the United States, the incidence of KD has been estimated recently by using hospital discharge data. An estimated 4,248 hospitalizations associated with KD occurred in the United States in the year 2000, with a median age of 2 years. The incidence of KD was highest among Asians and Pacific Islanders and lowest in white children. Boys outnumbered girls by 3:2, and 76% of children were younger than age 5 years. In-hospital mortality was 0.17%. Virtually all deaths in patients who have …