Kawasaki disease (KD) is characterized by marked activation of immune system and generalized
vasculitis including coronary
arteritis, and the intravenous gammaglobulin
therapy is recommended as the first line of the choices. However, in some fulminant cases the coronary
arteritis progresses rapidly and extensively in the early phase of the disease despite of the aggressive high-dose gammaglobulin administration. Recent observations indicated that the
vasculitis begins at the endothelial cells which are activated by several inflammatory
cytokines and are adhered to by
cytokine-activated cytotoxic immune cells. Thus, it is important for the prevention of coronary
arteritis and the resultant
coronary aneurysm to reduce the inflammatory
cytokines in the plasma. We reported here a 4-year-old girl with coronary
arteritis which began on the 3rd day of the disease. The patient was fulfilled the revised criteria of KD at the hospital admission. As the initial intravenous gammaglobulins (400 mg/kg/day for 3 consecutive days and the subsequent 1 g/kg single
intravenous infusion) revealed ineffective, which was judged by echocardiography in the persistence of brightness of the slightly widened coronary artery, and by laboratory data, we conducted therapeutic approach of
plasmapheresis (replacement of plasma with 5%
albumin in saline) on 8-10th days of KD.
Fever was down to normal range soon after the first
plasmapheresis, and after the second
pheresis CRP was reduced, and finally on the 10th day echocardiography demonstrated marked improvement of inflamed coronary artery. The precise mechanism of the effective
plasmapheresis is to be investigated, but it is possible that the removal of inflammatory
cytokines from the plasma ceased the subsequent endothelial cell damage and coronary
arteritis.(ABSTRACT TRUNCATED AT 250 WORDS)