Pathogenesis and treatment of Kawasaki's disease

Coronary artery damage resulting from Kawasaki's disease is the leading cause of acquired heart disease in children in the developed world. This review highlights advances in our understanding of the etiology of Kawasaki's disease, the immune response leading to vascular damage, potential biomarkers, and insights into mechanisms of disease addressed by an animal model. Clinical dilemmas are discussed in the context of the new American Heart Association recommendations for the diagnosis and treatment of Kawasaki's disease.

Improved understanding of the mechanisms of disease will assist in identifying predisposed individuals and in development of more effective therapy. Most investigators agree that an infectious trigger leads to massive activation of the immune system, resulting in a prolonged self-directed immune response at the coronary arteries. The etiology debate has centered on the nature of and mechanisms involved in immune activation. Genetic studies have not provided conclusive answers to these questions. Mechanistic studies done in animal models have pointed to specific biologic factors critical for coronary artery damage and together with studies in children may lead to more rationally conceived biologically based interventions. Increasingly, the questions regarding clinical management address timing of therapy and the management of children presenting with atypical Kawasaki's disease. New guidelines and management algorithms have been proposed by the American Heart Association to address these concerns.

Biochemical and phenotypic characterization of Kawasaki's disease continues to improve. Answers are closer on etiology, reliable biomarkers, valid predictors of coronary outcome, and improved treatment of this syndrome.