Abstract
Kawasaki disease (KD) is an acute-onset systemic vasculitis of medium-sized vessels that mostly affects infants and toddlers. Globally, it is the most common form of childhood primary vasculitis. Delayed diagnosis and treatment results in coronary artery aneurysms in up to 25% of all affected individuals. Thus, KD is the most common acquired heart disease in developed countries. Here, the current understanding of clinical presentations, pathophysiological concepts, disease-associated complications, and available pharmaceutical treatment is provided and discussed in the context of available literature.
Highlights
Kawasaki disease (KD) is a systemic vasculitis mostly affecting medium-sized arteries
A prospective clinical trial investigating effects of treatment with recombinant IL-1 receptor antagonis anakinra in KD patients with early coronary artery involvement (ANAKID trial), is not completed yet [75,76,77]
Regardless of anti-inflammatory effects, high-dose ASA alone does not reduce the risk of coronary aneurysms development, and should only be given in combination with IVIG and potentially other anti-iflamatory treatment options in KD
Summary
Kawasaki disease (KD) is a systemic vasculitis mostly affecting medium-sized arteries. Young patients under 12 months have an increased risk for the development of coronary artery aneurysms (up to 60% if untreated). Genetic variants in the transforming growth factor (TGF) pathway (TGFβ2, TGFβR2, SMAD3) are associated with an increased risk for the development of coronary aneurysms in European KD patients [15, 16]. This group of patients with “incomplete KD” exhibit a high risk for the development of complications, coronary artery aneurysms [34, 35].
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