Risk of coronary arterial lesions in immunoglobulin resistant Kawasaki disease

Objective: High-dose intravenous immunoglobulin (IVIG) decreases the risk for development of coronary arterial lesions (CAL) in Kawasaki disease (KD) while patients resistant to IVIG have higher risk. This study is aimed to investigate an additional therapy affects the risk for CAL in IVIG resistant KD. Design: The information from 11,510 children with KD was collected by the nationwide survey (2003-2004) in Japan. The secondary therapeutic strategies for IVIG resistant KD were an additional IVIG dose, steroids or both. The incidence of CAL was compared among 4 groups: G1, children responded to initial IVIG; G2, IVIG resistant patients receiving an additional IVIG dose; G3, IVIG resistant patients receiving additional steroids; G4, IVIG resistant patients receiving an additional IVIG dose plus steroids. CAL was assessed at 30th day of illness using ultrasound echocardiography. Results: 2229 patients (19.4%) were resistant to initial IVIG. Incidence of CAL was significantly lower in children responding to initial IVIG than in IVIG resistant patients (1.9% and 11.0%, respectively; odd’s ratio 6.3). The incidences of CAL in an each additional therapy group were as follows: G2: 6.7%, G3: 9.7%, G4: 22.2%. The risk for CAL was significantly higher in G4 as compared with G2 and G3. Giant aneurysmal formation (GA) was more frequently developed in G3 and G4 consisting of the patients receiving steroids (G1: 0.03%, G2: 0.63%, G3: 4.3%, G4: 3.7%). Conclusion: The incidence of CAL was considerably high in IVIG resistant KD and an additional therapy by steroids may increase the risk for GA.