Responsiveness to intravenous immunoglobulins and occurrence of coronary artery abnormalities in a single-centre cohort of Italian patients with Kawasaki syndrome.

Clues to predict the response to intravenous immunoglobulins (IVIG) and the development of coronary artery abnormalities (CAA) in children with Kawasaki syndrome (KS) are still undefined. We examined retrospectively the medical charts of children hospitalized between February 1990 and April 2009 with diagnosis of KS. A total of 32 Italian patients with a mean age of 23.8 months were analyzed and all received IVIG according to two schemes: 0.4 g/(kg day) for 5 days or 2 g/kg in a single infusion, combined with oral acetylsalicylic acid. General, clinical and laboratory data were registered. Each patient was evaluated with echocardiography at admission, then with 3-day and weekly frequency, respectively, during hospital stay and for the first 6-8 weeks since onset, and finally with a regular 6-12 month follow-up over time, according to patient risk stratification. Five patients showing significantly higher values of C-reactive protein (CRP) at admission were IVIG-resistant after the first infusion (P = 0.04) in comparison with the remaining 27. Five patients out of 32 developed CAA, with no statistical significance when analyzed for IVIG dosage or IVIG-resistance. The demonstration of CAA was significantly higher in children aged <12 months (P = 0.037). Our experience, limited to a single-center cohort of 32 patients with KS, though treated with two different IVIG schemes, has shown that higher values of CRP and younger age at onset are nodal points in determining, respectively, a failure in the response to IVIG and an increased occurrence of CAA.