Poor response to intravenous immunoglobulin in patients with a parental history of Kawasaki disease.

BACKGROUND

Patients whose parents had a history of Kawasaki disease (KD) often show a poor response to intravenous immunoglobulin (IVIG). There are very few reports describing the clinical characteristics and no reports on changes in C-reactive protein (CRP) level after IVIG administration in such cases.

METHODS

A single-center cohort study including 950 patients hospitalized for KD and treated with IVIG was conducted. The patients were divided into two groups: patients whose parents had a history of KD (PHK) (N = 16) and patients whose parents had no history of KD (NPHK) (N = 934). The clinical characteristics and responsiveness to IVIG were compared between the two groups.

RESULTS

The PHK group had higher CRP levels before administering IVIG than the NPHK group (P = 0.0010). CRP levels after IVIG administration were higher in the PHK group than in the NPHK group (P = 0.0094). The percentage of patients who received a second administration of IVIG on day 2 after the initial IVIG therapy was higher in the PHK group than in the NPHK group (P = 0.0016). The percentage of patients who required plasma exchange therapy in the PHK group was significantly higher than that in the NPHK group (P = 0.0010).

CONCLUSIONS

Patients in the PHK group had severe KD before IVIG administration, and these patients showed poor responsiveness to IVIG therapy.