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: Caroline Galeotti, Srini V Kaveri, Rolando Cimaz, Isabelle Koné-Paut, Jagadeesh Bayry
• Journal: Drug discovery today (Drug Discov Today) Vol. 21 Issue 11 Pg. 1850-1857 (11 2016) ISSN: 1878-5832 [Electronic] England
• PMID: 27506874 (Publication Type: Journal Article, Review, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2016 Elsevier Ltd. All rights reserved.

Topics

• Animals
• Disease Susceptibility
• Humans
• Mucocutaneous Lymph Node Syndrome (drug therapy, genetics, immunology)
• Risk Factors