Kawasaki disease (KD), formerly called mucocutaneous lymph node syndrome, is one of the most common vasculitides of childhood . 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 CA abnormalities, but the benefits in children who have already developed CA aneurysms 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 "Refractory Kawasaki disease" and "Kawasaki disease: Clinical features and diagnosis" and "Cardiovascular sequelae of Kawasaki disease".)
Theoretically, it should be possible to stratify therapy for Kawasaki disease (KD) according to disease severity defined by the likelihood of developing coronary artery (CA) aneurysms. While many risk scores have been proposed, none are 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 . Studies are ongoing to determine whether patients with risk factors for failing to respond to intravenous immune globulin (IVIG) treatment alone would benefit from more aggressive initial therapy for KD. (See "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 "Kawasaki disease: Clinical features and diagnosis" and "Refractory Kawasaki disease".)
A retrospective review of 195 patients with KD treated at four centers in the United States from 1981 to 2006 showed that application of the AHA/AAP guidelines resulted in the administration of IVIG therapy in almost all affected children (97 percent) .