Kawasaki disease is an acute febrile arteritis of childhood that can result in coronary artery aneurysms if untreated in the first 10 and ideally 7 days of illness. Kawasaki disease begins as a necrotizing arteritis with neutrophilic infiltrate, followed by subacute/chronic changes and luminal myofibroblastic proliferation that can cause coronary artery stenosis. Manifestations include the presence of ≥5 days of fever, together with clinical criteria of extremity changes, rash, conjunctivitis, oral changes, and unilateral cervical lymphadenopathy. Echocardiography should be performed at the time of diagnosis, then 1–2 weeks and 4–6 weeks later, with more frequent studies in individuals with coronary artery dilation or persistent fever. Coronary artery dimensions are characterized both as z-scores and absolute measurements, and coronary architecture evolves over time in children who have aneurysms in the first weeks of illness. Systematic follow-up and therapies are tailored to the degree of coronary disease and to coronary ischemia.
Medical therapies in patients with Kawasaki disease (KD) are administered to reduce the prevalence of coronary aneurysms, reduce systemic inflammation, and prevent coronary thrombosis. All patients with acute KD should be treated with intravenous immunoglobulin (IVIG) 2 g/kg, generally administered over 10–12 hours. Aspirin has never been shown to prevent aneurysms, but is given for its anti-inflammatory and antipyretic effects until the patient has been afebrile for ∼2 days, then lowered to an antiplatelet dose. Adjunctive therapy with a longer course of corticosteroids, together with IVIG and aspirin, may be considered for primary treatment in patients at high risk for development of aneurysms. For patients who have persistent or recrudescent fever after IVIG treatment without other explanation, adjunctive therapies include retreatment with IVIG, a tapering course of corticosteroids, infliximab, cyclosporine, cyclophosphamide, and other immunomodulatory therapies. Antithrombotic therapies are tailored to the risk of thrombosis, and range from aspirin alone for 4–6 weeks in children without aneurysms to a combination of anticoagulation and antiplatelet therapy for those with giant aneurysms.
Medical therapies in patients with Kawasaki disease (KD) are administered to reduce the prevalence of coronary aneurysms, reduce systemic inflammation, and prevent coronary thrombosis. All patients with acute KD should be treated with intravenous immunoglobulin (IVIG) 2 g/kg, generally administered over 10–12 hours. Aspirin has never been shown to prevent aneurysms, but is given for its anti-inflammatory and antipyretic effects until the patient has been afebrile for ∼2 days, then lowered to an antiplatelet dose. Adjunctive therapy with a longer course of corticosteroids, together with IVIG and aspirin, may be considered for primary treatment in patients at high risk for development of aneurysms. For patients who have persistent or recrudescent fever after IVIG treatment without other explanation, adjunctive therapies include retreatment with IVIG, a tapering course of corticosteroids, infliximab, cyclosporine, cyclophosphamide, and other immunomodulatory therapies. Antithrombotic therapies are tailored to the risk of thrombosis, and range from aspirin alone for 4–6 weeks in children without aneurysms to a combination of anticoagulation and antiplatelet therapy for those with giant aneurysms.
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