Antibodies against neutrophil cytoplasmic components in Kawasaki disease

The occurrence of antibodies against neutrophil cytoplasmic components in 39 children (23 boys, 16 girls, median age 2.0 years) with Kawasaki syndrome was studied. The conventional indirect immunofluorescence test (ANC-Ab) on alcohol-fixed neutrophils and two commercially available ELISA tests (ANCA-EIA and MPO-EIA) were employed to detect the antibodies. Fourteen (36%) of the 39 patients with Kawasaki disease had antibodies against neutrophil cytoplasmic components in at least one of the three tests used. Eleven patients were identified using the indirect immunofluorescence test. Five patients were positive in the MPO-EIA test and two additional patients in the ANCA-EIA test. The IF staining pattern was cytoplasmic in eight patients and perinuclear in three. The cytoplasmic staining pattern in patients with acute Kawasaki disease is different from that seen in patients with Wegener's granulomatosis. The occurrence of antibodies may assist in the diagnosis of some patients with Kawasaki disease, although neither the positivity itself nor the five different antibody profiles seem to have any other clinical relevance.