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Poor response to IVIG 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.