Management of Kawasaki disease in adults

Abstract

Kawasaki disease is the most common childhood vasculitis in the USA and the most common cause of acquired cardiac disease in children in developed countries. Since the vast majority of Kawasaki disease initially presents at <5 years of age, many adult cardiologists are unfamiliar with the pathophysiology of this disease. This vasculitis has a predilection for coronary arteries with a high complication rate across the lifespan for those with medium to large coronary artery aneurysms. An inflammatory cascade produces endothelial dysfunction and damage to the vascular wall, leading to aneurysmal dilatation. Later, pseudonormalisation of the vascular lumen occurs through vascular remodelling and layering thrombus, but this does not necessarily indicate resolution of disease or reduction of risk for future complications. There is a growing prevalence of Kawasaki disease, making it increasingly relevant for adult cardiologists as this population transitions into adulthood. As the 2017 American Heart Association (AHA) and 2014 Japanese Circulation Society (JCS) guidelines emphasise, Kawasaki disease requires rigorous follow-up with cardiac stress testing and non-invasive imaging to detect progressive stenosis, thrombosis and luminal occlusion that may lead to myocardial ischaemia and infarction. Due to differences in disease mechanisms, coronary disease due to Kawasaki disease should be managed with different pharmacological and non-pharmacological treatment algorithms than atherosclerotic coronary disease. This review addresses gaps in the current knowledge of the disease and its optimal treatment, differences in the AHA and JCS guidelines, targets for future research and obstacles to transition of care from adolescence into adulthood.

Adjuvant herbal therapy for targeting susceptibility genes to Kawasaki disease: An overview of epidemiology, pathogenesis, diagnosis and pharmacological treatment of Kawasaki disease

“…With the 1st suspected case reported in 1967 (Kawasaki, 2002), KD was identified as an acute pediatric systemic vasculitis with clinical symptoms such as sustained fever, oral mucosal lesions, pleural rash, swollen cervical lymph nodes, conjunctival hyperemia, severe swelling of hand and foot with skin peeling on fingertips. One of the most serious sequelae of KD is the inflammation of the heart arteries which lead to the formation of aneurysm and coronary artery lesions (CALs) (Denby et al, 2017). Besides, KD was reported to affect many different organ systems including blood vessels, mucous membranes, skins and lymph nodes (Kuwabara et al, 2015).…”

Cumulative incidence of Kawasaki disease with cardiac sequelae in Japan

“…However, the incidence of coronary events in people with a history of KD remains unclear. Japanese guidelines and AHA guidelines recommend several management options for each coronary sequela, but it is not known how frequently ischemic heart disease occurs among people with a history of KD‐related cardiac sequelae . Thus, the CIBC, which showed that one in 3,000 males and one in 6,000 females born in 2007 developed KD‐related cardiac sequela, is useful for considering their management to prevent ischemic heart disease in adulthood.…”

“…Patients with KD occasionally develop cardiac sequelae, increasing the risk of subsequent ischemic heart disease in adulthood . Investigating the proportion of individuals with KD with cardiac sequelae in the general population, especially by birth cohort, i.e.…”

A Review of Primary Vasculitis Mimickers Based on the Chapel Hill Consensus Classification

“…It can sometimes be seen in adults and presents with lymphadenopathy, hepatitis, arthralgia, constitutional symptoms, and coronary artery involvement. A long-term follow-up with a cardiologist is recommended to assess regularly for the onset of complications such as persistent coronary aneurysms and accelerated atherosclerosis [26,27]. Mimics of MVV are presented in Table 3. 3.1.…”