State-of-the-art acute phase management of Kawasaki disease after 2017 scientific statement from the American Heart Association

Abstract

Kawasaki disease (KD) has become the most common form of pediatric systemic vasculitis. Although patients with KD received intravenous immunoglobulin (IVIG) therapy, coronary arterial lesions (CALs) still occurred in 5%-10% of these patients during the acute stage. CALs may persist and even progress to stenosis or obstruction. Therefore, CALs following KD are currently the leading cause of acquired heart diseases in children. The etiology of CALs remains unknown despite more than four decades of research. Two unsolved problems are IVIG unresponsiveness and the diagnosis of incomplete KD. The two subgroups of KD patients with these problems have a high risk of CAL. In April 2017, the American Heart Association (AHA) updated the guidelines for the diagnosis, treatment, and long-term management of KD. Compared with the previous KD guidelines published in 2004, the new guidelines provide solutions to the aforementioned two problems and emphasize risk stratification by using coronary artery Z score systems, as well as coronary severity-based management and long-term follow-up. Therefore, in this study, we merged the AHA Scientific Statement in 2017 with recent findings for Taiwanese KD patients to provide potential future care directions for Taiwanese patients with KD.

Increased Incidence of Kawasaki Disease in Taiwan in Recent Years: A 15 Years Nationwide Population-Based Cohort Study

“…Determining whether this observation is the result of increased diagnosis due to improved awareness and/or access to specialist medical services or reflective of an actual increase in incidence is difficult (15). Both factors likely play a role in many countries, particularly in those countries where KD has been described more recently (16).…”

Decreased DNA methyltransferases expression is associated with coronary artery lesion formation in Kawasaki disease

“…We also used peripheral blood samples from KD patients before they underwent IVIG treatment (pre-IVIG) and then at least 3 days or 3 weeks after completing the IVIG treatment, as previously described in one of our previous studies 19. CAL was identified through echocardiography and defined as a coronary artery with an internal diameter of at least 3 mm (4 mm if the patient was more than 5 years old), a segment with an internal diameter at least 1.5 times larger than that of an adjacent segment, as 20, 21, or a Z score ≧ 2.5, and the severity of the coronary was classified using Z scores according to the 2017 AHA statement 22, 23. This study received approval from the Chang Gung Memorial Hospital's Institutional Review Board, and we also obtained written informed consent from the parents or guardians of all subjects.…”

Clinical observation of noncoronary cardiac abnormalities in Chinese children with Kawasaki disease

“…The diagnosis of KD depends on the principal clinical features, evidence of systemic inflammation and echocardiographic findings of CAA 25 . Inflammatory mediators play a vital role in judging the disease activity, clinical classification, IVIG response and CAA risk.…”

“…23,24 The diagnosis of KD depends on the principal clinical features, evidence of systemic inflammation and echocardiographic findings of CAA. 25 Inflammatory mediators play a vital role in judging the disease activity, clinical classification, IVIG response and CAA risk. In Korea, a retrospective study encompassing 795 KD patients by Ha et al 26 indicated that ANC was significantly higher in patients with CAA than in those without CAA, and CRP was significantly higher in complete KD patients than in incomplete KD patients.…”