Studies on the response of plasma lipids and lipoproteins to Kawasaki disease are scarce so far. The purpose of this study was to investigate the changes in plasma levels of lipids and apolipoproteins as well as the composition of different lipoproteins in patients during the acute and convalescence phases of Kawasaki disease. The results showed that during the acute phase, the concentrations of plasma high density lipoprotein (HDL)-cholesterol, apolipoprotein A-I (apoA-I) and A-II (apoA-II) were significantly reduced. While the reduction of HDL-cholesterol was mainly related to the lowering of esterified and unesterified cholesterols in HDL2 during the acute stage of Kawasaki disease, most of which recovered during the subsequent convalescence phase. The plasma concentration of triglycerides was 46% higher in patients during the acute phase of Kawasaki disease than in the control subjects, which may be ascribed to the increase of triglycerides in very low density lipoprotein (VLDL), low density lipoprotein (LDL) and HDL2. The variables studied above did not appear to be independent parameters. The level of plasma apoA-I showed a stronger negative association with triglyceride concentration (r = -0.22) than apoA-II (r = -0.11) and HDL-cholesterol (r = -0.07). Furthermore, the levels of cholesterol, apoA-I and apoA-II in HDL2, but not in HDL3, were inversely correlated with the levels of triglyceride. We conclude that the temporary changes of lipid levels associated with Kawasaki disease results predominantly from alterations of lipoprotein composition.