Will aggressive initial therapy benefit kawasaki disease
Are there patients with KD who might benefit from more aggressive initial therapy?
Numerous studies have shown that 10%–35% of patients with KD fail to respond or develop recrudescent fever. These patients are characterized as IVIG-resistant and are at high risk for developing coronary artery aneurysms. They, therefore, require additional therapy to interrupt the inflammatory process. There have been many efforts to prospectively identify KD patients who have a high likelihood of being IVIG-resistant or who will develop coronary artery aneurysms in order to treat these patients more aggressively and decrease the incidence of coronary artery lesions. Prior studies from Japan have resulted in successful scoring systems including the Egami, Sano, and Kobayashi scores to predict IVIG resistance. Intensified therapy with prednisolone and IVIG in these high-risk patients improved coronary artery outcomes in the study populations. Unfortunately, these scoring systems have low sensitivity in heterogeneous US populations, and efforts to identify high-risk, IVIG-resistant patients in North American cohorts have been unsuccessful. Although rigorous, prospectively controlled studies are lacking, some US centers have taken the approach of targeting KD patients with intensified therapy who present with coronary artery lesions or who have epidemiologic risk factors such as young age. Intensified regimens include the addition of infliximab or steroids to standard therapy of IVIG and aspirin.
