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Kawasaki disease: insights into pathogenesis and approaches to treatment
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Journal Article
Kawasaki disease: insights into pathogenesis and approaches to treatment
2015
Overview
Key Points
Epidemiologic data strongly suggest an infectious aetiology for Kawasaki disease, which is the leading cause of acquired heart disease among children in developed countries
Necrotizing arteritis, subacute chronic vasculitis, and luminal myofibroblastic proliferation are three linked processes underlying the arteriopathy associated with Kawasaki disease
Genetic susceptibility is indicated by the strikingly high rate of Kawasaki disease in children of Asian ethnicity and by its increased incidence in first-degree relatives of affected patients
Timely diagnosis and treatment of Kawasaki disease with intravenous immunoglobulin (IVIg) and aspirin substantially decreases the risk of developing coronary artery abnormalities
Adjunctive therapy with corticosteroids is of value in Japanese patients at particularly high risk of coronary complications, but identification of such high-risk patients is difficult in ethnically diverse populations
Management of patients who do not respond to standard therapy is challenging; options include pulsed steroids, additional IVIg, and infliximab or other immunomodulatory agents
Considerable progress has been made in understanding the pathologic processes and pathophysiology of Kawasaki disease. This article also discusses genetic susceptibility to Kawasaki disease and describes current approaches to treatment of the acute stage of the disease.
This Review summarizes recent advances in understanding of the pathologic processes and pathophysiologic mechanisms leading to coronary arteritis in Kawasaki disease, and describes current approaches to its treatment. Kawasaki disease is the most common cause of acquired heart disease among children in developed countries, in whom the resulting coronary artery abnormalities can cause myocardial ischaemia, infarction and even death. Epidemiologic data strongly suggest an infectious aetiology, although the causative agent has yet to be identified. Genetic factors also increase susceptibility to Kawasaki disease, as indicated by its strikingly high incidence rate in children of Asian ethnicity and by an increased incidence in first-degree family members. The treatment of Kawasaki disease is based on timely administration of intravenous immunoglobulin and aspirin. However, the management of patients who do not respond to this standard therapy remains challenging; although several options are available, comparative data on which to base treatment decisions are scarce. The added value of adjunctive therapy with corticosteroids in patients at particularly high risk of coronary complications has been demonstrated in Japanese populations, but identification of high-risk patients has proven to be difficult in ethnically diverse populations.
Publisher
Nature Publishing Group UK,Nature Publishing Group
