INTRODUCTION

Kawasaki disease (KD), formerly called mucocutaneous lymph node syndrome, is one of the most common vasculitides of childhood [1]. It is typically a self-limited condition with fever and manifestations of acute inflammation lasting for an average of 12 days without therapy. However, cardiovascular complications, particularly coronary artery (CA) aneurysms, which can lead to occlusion and cardiac ischemia, may develop and lead to significant morbidity and mortality. (See "Cardiovascular sequelae of Kawasaki disease".)

The frequency of CA aneurysm development, and associated morbidity and mortality, have been dramatically decreased as a result of intravenous immune globulin (IVIG) therapy. This therapy is most effective for preventing coronary artery abnormalities, but the benefits in children who have already developed CA are more equivocal. Thus, expeditious diagnosis and timely treatment are critical to achieve the optimal clinical outcome. The initial treatment of KD is discussed in this review. The treatment of refractory KD, diagnosis, clinical manifestations, and cardiovascular sequelae are reviewed elsewhere. (See "Treatment of refractory Kawasaki disease" and "Clinical manifestations and diagnosis of Kawasaki disease" and "Cardiovascular sequelae of Kawasaki disease".)

OVERVIEW

Theoretically, it should be possible to stratify therapy for KD according to disease severity defined by the likelihood of developing CA aneurysms. While many risk scores have been proposed, none have been validated across different populations [2,3]. Since no criteria have been developed that can reliably identify children most at risk for severe disease at the time of initial presentation, all children diagnosed with KD or incomplete KD are treated at the time of diagnosis [1]. (See "Treatment of refractory Kawasaki disease", section on 'Risk factors'.)

In 2004, guidelines by the American Heart Association (AHA) and the American Academy of Pediatrics (AAP) were developed for the treatment of patients who fulfill the diagnostic criteria for KD (table 1) and for those who do not (so-called incomplete KD) (algorithm 1) [4,5]. The recommended initial therapy includes IVIG (2 gm/kg) administered as a single infusion over 8 to 12 hours and aspirin (initial dose of 80 to 100 mg/kg daily divided into four doses). Additional agents are used only for children who fail to respond to standard therapy. (See "Clinical manifestations and diagnosis of Kawasaki disease" and "Treatment of refractory Kawasaki disease".)

A retrospective review of 195 patients with KD treated at four centers in the United States from 1981 to 2006 showed application of the AHA/AAP guidelines would result in the administration of IVIG therapy in almost all affected children (97 percent) [6].