The Value Of Steroid Treatment For The Intravenous Immunoglobulin Unresponsive Kawasaki Disease

ObjectiveTo investigate the value of steroid treatment for intravenous immunoglobulin(IVIG)unresponsive Kawasaki disease(KD).MethodsThe clinical data of 143 children with initial IVIG unresponsive KD was collected.All cases were divided into 3 groups: IVIG retreatment group(using an additional IVIG in 107 cases),steroid group(oral prednisone or intravenous methylprednisolone sequentially oral prednisone in 12 cases),IVIG retreatment plus steroid group(clinical symptoms did not relieve after IVIG retreatment and then treated with steroid in 24 cases).The clinical data of 3 groups were analyzed retrospectively.The echocardiography data was also followed up to evaluate coronary outcomes.In order to study the effect of steroid usage mode on clinical manifestations in acute stage and coronary outcomes,steroid group and IVIG retreatment plus steroid group were divided into oral steroid group and intravenous methylprednisolone sequentially oral prednisone group(intravenous-oral steroid group).ResultsThere were no significant difference in duration of fever after treatment,decreasing the percentage of neutrophils(N%)and the incidence of coronary artery dilatation(CAD)in acute stage in the IVIG retreatment group compared with the IVIG retreatment plus steroid group(P > 0.05).While IVIG retreatment tended to shorten the whole fever time and reduce white blood cell(WBC)counts significantly(P =0.015).There was a significant reduction in the C-reactive protein(CRP)and elevation in the platelet(PLT)counts after IVIG retreatment plus steroid therapy.The study showed a higher incidence of coronary artery aneurysm(CAA)in acute stage and CAD in half a year later in the IVIG retreatment plus steroid group compared to the IVIG retreatment group,but there was no difference in the incidence of coronary artery lesions(CALs)after a year(P > 0.05).There were no significant differences in duration of fever after treatment,decreasing N% and CRP,elevating PLT counts,the incidence of CAA in acute stage and long-term progression of coronary artery(P > 0.05)in the IVIG retreatment group compared with the steroid group.The shorter time of the whole fever and lower WBC counts were observed in the IVIG retreatment group compared with the steroid group.The IVIG retreatment group had higher incidence of CAD in acute stage than the steroid group(52.8% vs 16.7%,P=0.029).The steroid group showed no significant differences in the whole fever time,duration of fever after treatment,elevating WBC counts,decreasing N% compared with the IVIG retreatment plus steroid group(P > 0.05).But CRP decreased and PLT increased significantly in IVIG retreatment plus steroid group(P = 0.010).The steroid group had a lower incidence of CALs in the first week after treatment(P < 0.05)compared with the IVIG retreatment plus steroid group,but there were no significant difference half a year later.The results showed in the oral ordinary dose prednisone group,there were no significant differences in the whole fever time,duration of fever after treatment,elevating WBC counts,decreasing N% and CRP levels and the incidence of CALs in acute stage and one year later(P > 0.05),compared with that in the intravenous infusion ordinary dose methylprednisolone sequentially oral prednisone group.But the PLT counts significantly increased in intravenous-oral steroid group(P = 0.010).ConclusionThis study suggests that direct application of steroid treatment is effective and relatively safe for initial IVIG unresponsive KD patients,and the incidence of CALs does not increase in long-term follow-up.Steroid rescue treatment for those who showed no response to additional IVIG does not affect the long-term prognosis of coronary artery.Oral ordinary dose steroid or intravenous way showed no significant difference on the effect of the treatment in acute period and long-term prognosis for IVIG unresponsive KD patients.Intravenous way significantly increase PLT level which results in an increased thrombotic risk.Therefore,taking oral steroid maybe the better choice.Multi-center,randomized,double blind,large sample prospective study should be taken to figure out when the steroid should be chosen: after initial IVIG or additional IVIG unresponsiveness.