Abstract
Kawasaki disease is an acute vasculitis with a particular involvement of the coronary arteries. Coronary artery aneurysms develop in 20% of untreated children. It has been shown that early treatment with intravenous immunoglobulins and aspirin decreases this risk to 5%, but the medium to long term prognosis of children with Kawasaki disease is still unclear. To determine the outcome of the disease and risk factors for poor evolution, we reviewed retrospectively the medical records of all patients with a diagnosis of Kawasaki disease at our Institution between 1981 and 2014. Among the 207 patients included in the study, 96 patients had coronary diameter anomalies (46.4%) at diagnosis and children with atypical ages for Kawasaki disease (<1 year or >10 year of age) were more often affected with aneurysms or dilatations. Eighty-four of them had complete regression of coronary aneurysms during the follow-up (87.5%) Absence of immunoglobulins in the acute phase was associated with less regression rate (57.1 vs. 92.2%), and boys had greater z-scores at last echocardiography, statistically significant for the left anterior descending artery. We found rare complications after the acute phase documented in our patient charts (only 3.8%). Recurrence of the disease occurred in 5 children (2.4%) and myocardial ischemia in 3 patients (1.4%), all with initial coronary aneurysm.Conclusion: Medium to long term prognosis after Kawasaki disease is excellent. Boys, patients not treated with immunoglobulins or outside the usual age range are more at risk for an unfavorable outcome.
Highlights
Kawasaki disease (KD) is an acute systemic vasculitis of early childhood considered the leading cause of acquired heart disease in children in developed countries
Cardiac lesions are a hallmark of KD and coronary artery aneurysms (CAA) develop in 20% of untreated children and can lead to coronary stenosis, myocardial infarction (MI), or sudden death
The majority of children received treatment before the 7th day of illness, but there were still 22.7% of patients with a late therapy due to late diagnosis. This rate in delayed treatment is similar to other studies, but it is crucial to improve diagnosis and early therapy as we found that intravenous immunoglobulin (IVIG) treatment during the acute phase is associated with greater regression rate and, with less cardiac sequelae
Summary
Kawasaki disease (KD) is an acute systemic vasculitis of early childhood considered the leading cause of acquired heart disease in children in developed countries. Cardiac lesions are a hallmark of KD and coronary artery aneurysms (CAA) develop in 20% of untreated children and can lead to coronary stenosis, myocardial infarction (MI), or sudden death. Pericarditis complicated by cardiac tamponade or myocarditis associated with myocardial dysfunction can occur during the acute phase [1, 2]. Despite almost 50 years of research, the etiology of KD remains unknown. An infectious trigger which causes an excessive inflammatory response in genetically predisposed children is a widespread hypothesis, but no specific pathogen has been identified yet [3]. Frontiers in Pediatrics | www.frontiersin.org de La Harpe et al
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