The Immunologic Cascade of Kawasaki Syndrom Trigerred by An Infectious Agent?

Kawasaki syndrome (KS) is the most common cause of acquired heart disease in children. This acute, self-limited vasculitis results in permanent coronary artery damage in up to 25% of untreated children. High dose intravenous gamma globulin reduces the risk of coronary artery aneurysm to 3-5% if administered early in the course of disease. However, without a specific diagnostic test, affected children may be difficult to recognize, and delayed diagnosis and treatment continue to result in potentially preventable morbidity and mortality. The etiology of Kawasaki syndrome (KS) remains unknown despite 30 years of intensive search for an agent.
Associations have been observed between antecedent respiratory illness and KS. It is proposed that an agent causing KS could first infect the upper respiratory tract before triggering a systemic immunologic response. Researchers found an increased IgA plasma cell infiltration of the upper respiratory tract and coronary arteries in KS patients, therefore, further support the respiratory route as a potential portal of entry for the causative agent. The presence of IgA-secreting plasma cells in the upper respiratory tract mimics the response seen in autopsies of children who died of known viral respiratory infection such as respiratory syncytial virus.
(Pediatr Infect Dis J. 2008;27(11):981-985)