title: Predisposing factors, pathogenesis and therapeutic intervention of Kawasaki disease.
abstract: Kawasaki disease (KD) is an acute febrile childhood inflammatory disease, associated with coronary artery abnormalities. The disease is believed to result from an aberrant inflammatory response to an infectious trigger in a genetically predisposed individual. KD is associated with an endothelial cell injury as a consequence of T cell activation and cytotoxic effects of various proinflammatory cytokines. Intravenous immunoglobulin (IVIG) infusion and aspirin are the standard treatment of acute KD. However, 10-20% of patients show resistance to IVIG therapy and present higher risk of coronary vasculitis. The relative roles of second IVIG infusion, corticosteroids, calcineurin inhibitors, interleukin-1 antagonists and anti-tumor necrosis factor agents remain uncertain. In this review, we highlight the predisposing factors, pathogenesis and therapeutic intervention of KD, particularly new therapeutics for IVIG-resistant patients.
authors: Institut National de la Santé et de la Recherche Médicale Unité 1138, Paris F-75006, France; Sorbonne Universités, UPMC Univ Paris 06, UMR S 1138, Paris F-75006, France; Centre de Recherche des Cordeliers, Equipe - Immunopathology and Therapeutic Immunointervention, Paris F-75006, France; Department of Pediatric Rheumatology, National Referral Centre of Auto-inflammatory Diseases, CHU de Bicêtre, le Kremlin Bicêtre, University of Paris Sud, F-94270, France.
authors: Caroline
authors: C
authors: Galeotti
authors: Institut National de la Santé et de la Recherche Médicale Unité 1138, Paris F-75006, France; Sorbonne Universités, UPMC Univ Paris 06, UMR S 1138, Paris F-75006, France; Centre de Recherche des Cordeliers, Equipe - Immunopathology and Therapeutic Immunointervention, Paris F-75006, France; Université Paris Descartes, Sorbonne Paris Cité, UMR S 1138, Paris F-75006, France.
authors: Srini V
authors: SV
authors: Kaveri
authors: Anna Meyer Children's Hospital and University of Florence, Florence, Italy.
authors: Rolando
authors: R
authors: Cimaz
authors: Department of Pediatric Rheumatology, National Referral Centre of Auto-inflammatory Diseases, CHU de Bicêtre, le Kremlin Bicêtre, University of Paris Sud, F-94270, France.
authors: Isabelle
authors: I
authors: Koné-Paut
authors: Institut National de la Santé et de la Recherche Médicale Unité 1138, Paris F-75006, France; Sorbonne Universités, UPMC Univ Paris 06, UMR S 1138, Paris F-75006, France; Centre de Recherche des Cordeliers, Equipe - Immunopathology and Therapeutic Immunointervention, Paris F-75006, France; Université Paris Descartes, Sorbonne Paris Cité, UMR S 1138, Paris F-75006, France. Electronic address: jagadeesh.bayry@crc.jussieu.fr.
authors: Jagadeesh
authors: J
authors: Bayry
mesh_terms: D000818:Animals; D004198:Disease Susceptibility; D006801:Humans; D009080:Mucocutaneous Lymph Node Syndrome / Q000188:drug therapy / Q000235:genetics / Q000276:immunology; D012307:Risk Factors