Kawasaki disease (mucocutaneous lymph node syndrome) has emerged as a major pediatric disorder throughout the developed world, including the United States where it is now a leading cause of acquired heart disease in children. Coronary artery abnormalities, including ectasia, aneurysms, stenosis, and thrombosis, that may result in myocardial ischemia and/or infarction, develop in approximately 20-25% of patients as a consequence of coronary arteritis. Although epidemiologic and clinical findings strongly suggest an infectious etiology, the etiology of Kawasaki disease remains unknown. Marked immune activation is present in the acute state of Kawasaki disease, Investigators in Japan and a U.S. multicenter investigative group have demonstrated in controlled studies that administration of IVGG early in the course of Kawasaki disease is associated with (i) a striking anti-inflammatory effect, and (ii) a marked reduction in the development of coronary abnormalities, as assessed by echocardiography and/or angiography. The mechanism(s) by which IVGG produces these dramatic effects is unclear. Possible mechanisms include (i) Fc receptor blockade, (ii) a direct anti-etiologic agent (neutralization) effect, (iii) an anti-toxic effect, (iv) an immunomodulating effect possibly mediated either by anti-idiotypic antibodies or by induction of suppressor T cells, and (v) down-regulation of cytokine production by activated immune cells. Clarification of the mechanism of action of IVGG in Kawasaki disease should provide insights into the pathogenesis and/or the etiology of this fascinating disorder.
ASJC Scopus subject areas
- Immunology and Allergy
- Pathology and Forensic Medicine