Pathogenesis and treatment of intravenous immunoglobulin-resistant Kawasaki disease

Kawasaki disease(KD) is an acute, self-limited vasculitis of childhood and has become the lea-ding cause of acquired pediatric heart disease.The underlying etiology remains unknown.The disease itself may be the characteristic manifestation of a common pathway of immune-mediated vascular inflammation in susceptible hosts.Intravenous immunoglobulin (IVIG) is now widely accepted as the first-line therapy for KD.However, approximately 10%-20% of patients are resistant to IVIG.Although, an additional administration of IVIG is often chosen for the treatment of KD patients resistant to the first administration of IVIG, its efficacy is reported to be lower than that of the first IVIG dose.Thus, it is clear that some KD patients can not be treated successfully by IVIG alone, even if it is used repetitively.Currently, methylprednisolone(MP) is used for the treatment of IVIG-resistant KD.However, the proper use of MP for maximum effect and safety has not yet been elucidated.Tumor necrosis factor-α blocker, infliximab effective in the control of inflammation in patients with resistant KD.Cyclosporin treatment is also a promising option for patients with refractory KD.Plasma exchange was a safe, effective prophylactic measure against coronary artery lesions in children with KD refractory to intravenous gamma globulin therapy. Key words: Kawasaki disease; Intravenous immunoglobulin-resistant; Pathogenesis; Treatment