Abstract 46: Effect Of Infliximab And Plasma Exchange Therapy On Activated Monocytes/macrophages In Kawasaki Disease Intractable To IVIG.

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Background: Kawasaki disease (KD) is a generalized vasculitis of unknown etiology that occurs predominantly in infant and children. KD has been reported as one of the cytokine-induced disease. The monocyte / macrophage is thought to the subject of proinflammatory cytokines sources. It has been reported that IVIG therapy in KD normalizes the increased numbers of activated CD14 + CD16 + monocyte / macrophage. We examined the effect of the infliximab (IFX) and plasma exchange (PE) therapy on the peripheral blood cells, especially the monocyte / macrophage in KD intractable to intravenous immunoglobulin (IVIG). Patients: We studied 9 patients with KD intractable to IVIG (2g/kg/dose), seven with IFX therapy (5mg/kg/dose), four with PE therapy (including two patients of refractory to IFX therapy), who were seen at our hospital. The median age and body weight were 3.3 years (range, 0.5 to 5.5 years) and 13.0kg (range, 6.1 to 20.3kg), respectively. White blood cell count and differentiation were compared before with after the treatment. Results: IFX group: White blood cell count was significantly decreased to 11640 ± 3509 from 14141 ± 4673/μl. The neutrophil count was decreased and the lymphocyte count was increased. Both the counts were normalized. CD14 + monocyte / macrophage count (normal value: 339 ± 91/μl) was significantly decreased to 488 ± 195 from 999 ± 528/μl. Especially, the activated CD14 + CD16 + monocyte / macrophage count (normal value: 35 ± 18/μl) was significantly decreased to 69 ± 45 from 176 ± 164/μl. PE group: White blood cell count was decreased to 10683 ± 4665 from 16810 ± 2527/μl. Neutrophil count was decreased, but the lymphocyte count was no changed. CD14 + monocytes / macrophage count was decreased to 401 ± 278 from 1402 ± 1286/μl. The activated CD14 + CD16 + monocyte / macrophage count was decreased to 24 ± 9 from 243 ± 364/μl. Conclusion: In KD intractable to IVIG, the CD14 + CD16 + monocyte / macrophage count of prior to the treatment was increased. Both IFX and PE therapy has the effects of decreasing the CD14 + CD16 + monocyte / macrophage count which involved in the pathogenesis of KD.