Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

Abstract

BACKGROUND:Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries. METHODS AND RESULTS:To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and longterm outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up. CONCLUSIONS:These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances. K awasaki disease (KD) is an acute, self-limited febrile illness of unknown cause that predominantly affects children <5 years of age. When initially described, the potential for coronary artery complications was not appreciated. KD is now the most common cause of acquired heart disease in children in developed countries. In the absence of pathognomonic tests, the diagnosis continues to rest on the identification of principal clinical findings and the exclusion of other clinically similar entities with known causes. Timely initiation of treatment with intravenous immunoglobulin (IVIG) has reduced the incidence of coronary artery aneurysms defined from absolute luminal dimensions from 25% to ≈4%. Ongoing studies with additional therapies have not substantially reduced this residual risk. The long-term prognosis is determined by the initial and current level of coronary artery involvement. Certain subsets of patients are at risk for myocardial ischemia from coronary artery thrombosis and stenoses. Medical management of such patients hinges on judicious use of thromboprophylaxis and vigilance to identify evolving stenoses. Invasive revascul...

Recent advances in the diagnosis of Kawasaki disease

“…The two KD groups (incomplete and complete KD) included a total of 343 children who were diagnosed with KD and treated with IVIg at Chonnam National University Hospital between December 2007 and March 2016. Complete KD was diagnosed according to American Heart Association criteria suggested in 2017 [5]. The criteria include fever persisting for at least 5 days and fulfillment of at least four of five clinical symptoms [5].…”

“…Complete KD was diagnosed according to American Heart Association criteria suggested in 2017 [5]. The criteria include fever persisting for at least 5 days and fulfillment of at least four of five clinical symptoms [5]. In contrast, incomplete KD was diagnosed when a patient presented three or fewer symptoms and other possible causes of the fever were excluded [5].…”

“…The criteria include fever persisting for at least 5 days and fulfillment of at least four of five clinical symptoms [5]. In contrast, incomplete KD was diagnosed when a patient presented three or fewer symptoms and other possible causes of the fever were excluded [5]. The control group included 62 age-matched children who visited our center for an evaluation of febrile illness and underwent laboratory testing for NT-proBNP; we excluded those with heart diseases such as congenital heart disease, cardiomyopathy, and acute myocarditis, which can affect NT-proBNP levels [9].…”

“…Therefore, in cases of vague clinical symptoms, the diagnosis inevitably depends on adjuvant diagnostic markers. Some that are already broadly adopted are elevated C-reactive protein (CRP), elevated erythrocyte sedimentation rate (ESR), low albumin, anemia, elevation of alanine aminotransferase (ALT), a high platelet count after 7 days, and a high white blood cell count or pyuria [5].…”

Afebrile Kawasaki Disease Presenting as Intestinal Pseudo-Obstruction

“…The diagnosis is based on clinical criteria and non- (1). These clinical features can vary and manifest sequentially which makes the diagnosis difficult at one point of the presentation.…”