[Evaluation of unresponsiveness to standard high-dose gamma globulin therapy in Kawasaki disease]

We investigated the incidence of unresponsiveness to intravenous gamma globulin (IVIG) treatment in Kawasaki disease (KD) and evaluated its relation with coronary artery involvement.

The study included 20 children (13 boys, 7 girls; mean age 4.2+/-3.4 years; range 9 months to 12 years) with KD. The mean disease duration on admission was 7.3+/-2.4 days (range 5 to 14 days). Initial treatment consisted of a single dose of IVIG and high-dose of aspirin. Unresponsiveness was defined as the persistence of fever and other symptoms within the first 48 hours of treatment. All the patients were evaluated by two-dimensional echocardiography before and after treatment. The mean follow-up period was 16.5+/-2.8 months (range 9 to 24 months).

Unresponsiveness was seen in five patients (25%), who received a subsequent dose of IVIG, which improved fever in two patients. The remaining three patients received high-dose methylprednisolone. One patient who showed no response to either IVIG or methylprednisolone was treated with low-dose oral methotrexate. Six patients (30%) had coronary artery involvement (4 dilatations, 2 aneurysms), five patients on admission echocardiography, and one patient on control echocardiography seven days after treatment. Of five unresponsive patients, four (80%) had coronary artery involvement on admission. Patients with coronary involvement underwent coronary angiography after a mean of one year. Five patients had normal coronary arteries, whereas no angiographic regression was observed in one patient who had a giant coronary artery aneurysm on admission and was treated with oral methotrexate.

The incidence of unresponsiveness to treatment was markedly high in KD patients who had coronary artery involvement on admission.