title: Management of acute and refractory Kawasaki disease.
abstract: Acute Kawasaki disease (KD) is treated with high-dose intravenous immunoglobulin (IVIG), which is proven to decrease the incidence of coronary artery aneurysms from 25% to less than 5%. Aspirin is also given, although the evidence base is less secure. There is increasing evidence for steroid therapy as adjunctive primary therapy with IVIG, especially in Asian children. Approximately 10-30% of patients fail to respond to the initial IVIG and are at increased risk of coronary artery aneurysms. The optimal treatment for IVIG-nonresponsive KD remains controversial. Management options include further dose(s) of IVIG, corticosteroids, TNF-α blockade, cyclosporin A, anti-IL-1 and anti-CD20 therapy. In this article, the authors review the current evidence for treatment of acute KD and discuss options for IVIG nonresponders.
authors.affiliation: Department of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Center, H7-230, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
authors.forename: Carline E
authors.initials: CE
authors.lastname: Tacke
authors.forename: David
authors.initials: D
authors.lastname: Burgner
authors.forename: Irene M
authors.initials: IM
authors.lastname: Kuipers
authors.forename: Taco W
authors.initials: TW
authors.lastname: Kuijpers
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001241:Aspirin / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D003323:Coronary Aneurysm / Q000209:etiology / Q000517:prevention & control; D016572:Cyclosporine / Q000627:therapeutic use; D004791:Enzyme Inhibitors / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use*; D008727:Methotrexate / Q000627:therapeutic use; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications / Q000188:drug therapy*; D000069283:Rituximab; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors
chemical_list: D000305:Adrenal Cortex Hormones; D000894:Anti-Inflammatory Agents, Non-Steroidal; D058846:Antibodies, Monoclonal, Murine-Derived; D004791:Enzyme Inhibitors; D016756:Immunoglobulins, Intravenous; D014409:Tumor Necrosis Factor-alpha; D000069283:Rituximab; D016572:Cyclosporine; D001241:Aspirin; D008727:Methotrexate