Abstract
More than 40 years have passed since Kawasaki syndrome (KS) was first described. Yet KS still remains an enigmatic illness which damages the coronary arteries in a quarter of untreated patients and is the most common cause of childhood-acquired heart disease in developed countries. Many gaps exist in our knowledge of the etiology and pathogenesis of KS, making improvements in therapy difficult. In addition, many KS features and issues still demand further efforts to achieve a much better understanding of the disease. Some of these problem areas include coronary artery injuries in children not fulfilling the classic diagnostic criteria, genetic predisposition to KS, unpredictable ineffectiveness of current therapy in some cases, vascular dysfunction in patients not showing echocardiographic evidence of coronary artery abnormalities in the acute phase of KS, and risk of potential premature atherosclerosis. Also, the lack of specific laboratory tests for early identification of the atypical and incomplete cases, especially in infants, is one of the main obstacles to beginning treatment early and thereby decreasing the incidence of cardiovascular involvement. Transthoracic echocardiography remains the gold-standard for evaluation of coronary arteries in the acute phase and follow-up. In KS patients with severe vascular complications, more costly and potentially invasive investigations such as coronary CT angiography and MRI may be necessary. As children with KS with or without heart involvement become adolescents and adults, the recognition and treatment of the potential long term sequelae become crucial, requiring that rheumatologists, infectious disease specialists, and cardiologists cooperate to develop specific guidelines for a proper evaluation and management of these patients. More education is needed for physicians and other professionals about how to recognize the long-term impact of systemic problems related to KS.
Highlights
Kawasaki syndrome (KS, OMIM 611775) is an acute necrotizing vasculitis of the medium and small-sized vessels, predominantly occurring in children aged 6 months-5 years, with a male-to-female ratio of 1.5-1.8 to 1 and a life-threatening predisposition to involve coronary arteries [1]
The current treatment strategy is based on intravenous immunoglobulins (IVIG) therapy (2 g/kg of body weight in a 10-12 hour-infusion) and aspirin (50-100 mg/kg daily, divided in four doses), both given within the tenth day of fever onset [37]
Vaccinations in children with Kawasaki syndrome Routine vaccinations are recommended for all individuals with KS
Summary
Kawasaki syndrome (KS, OMIM 611775) is an acute necrotizing vasculitis of the medium and small-sized vessels, predominantly occurring in children aged 6 months-5 years, with a male-to-female ratio of 1.5-1.8 to 1 and a life-threatening predisposition to involve coronary arteries [1]. Atypical and incomplete KS raise criticism about the validity and suitability of diagnosis based on the classic clinical criteria These patients with incomplete forms of the disease present the coronary artery involvement, but these children may clinically display noncoronary cardiac lesions, such as pancarditis, conduction system abnormalities, and subclinical ventricular dysfunction or subtle ventricular dilations, all involvements possibly independent of CAA [33]. Therapy of primary Kawasaki syndrome Since no criteria have been developed that can reliably identify children at higher risk for severe disease at the time of initial presentation, all children diagnosed with KS must be treated at the time of diagnosis Since both KS etiology and pathogenesis are not completely clarified, the general aims of treatment are to rapidly diminish the inflammation within the vascular system and in the coronary arteries, minimize the incidence and progression of CAA, and prevent arterial thrombosis by inhibiting platelet aggregation [36]. Recent studies evaluating vaccinations side effects minimize the risk that vaccines trigger KS, though a few isolated relationships have been reported for hepatitis B and rotavirus live vaccines [75,76,77]
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