Kawasaki disease (KD) is an acute vasculitis affecting previously healthy young infants and children. During its first acute stage, it manifests as a high fever (over 101°F) that persists for at least five days (Patel & Shulman, 2015). Furthermore, four of the following clinical characteristics are required to diagnose KD: “bilateral conjunctival injection; erythema of the oropharynxwith red, cracked lips and ‘strawberry tongue’; oedema anderythema of hands and feet; erythematous rash; and non-suppurative unilateral cervical lymph node enlargement>1-5cmin diameter” (Patel & Shulman, 2015, p. 620). The subsequent subacute phase, which occurs 10 to 25 days after the onset of acute symptoms, is “often characterized by periungual desquamation of thefingers andtoes and thrombocytosis” (Patel & Shulman, 2015, p. 620). If untreated, a high risk of sudden death exists for patients with aneurysms (Patel & Shulman, 2015). Due to this risk, timely diagnosis and subsequent support of patients with KD is crucial.
KD primarily affects children between 6 months and 5 years, but can occur in younger infants and adolescents. It can occur in all ethnic groups with the highest incidence in those of Asian background (Patel & Shulman, 2015). The illness is most prevalent in Japan with an annual incidence of 264.8 per 100 000 children under 5 years of age in 2012 (McCrindle et al., 2017, p. e928). It is significantly less common in the continental U.S., where an estimated incidence rate is “≈25 per 100 000 children <5 years of age” (McCrindle et al., 2017, p. e928). Furthermore, it is significantly more likely to affect boys than girls (Patel & Shulman, 2015; McCrindel et al., 2017). Thus, a clear demographic disparity exists in the incidence of KD.
When diagnosing KD, it is important to note that the disease is relatively uncommon, its exact causes are unknown and no specific test exists for it. Therefore, differentiating it from other diseases that may represent similar symptoms can be challenging, but important. Measles, for instance, exhibits similar clinical features and should be considered if the patient is not immunized (McCrindle et al., 2017). Furthermore, laboratory tests, such as pleocytosis in an infant’s cerebrospinal fluid can lead to KD being mistaken for aseptic meningitis (McCrindle et al., 2017). Because of these factors, KD can be challenging to diagnose specifically and treat. Furthermore, as different clinical characteristics can be interpreted differently by different specialists, collaborating during the differential diagnosis is crucial to confirming this diagnosis.
Treatment of KD is based on the prevention of cardiovascular complications, particularly aneurysms. The primary method of reducing the incidence of such complications is the administration of intravenous immunoglobulin (IVIG) (Eleftheriou, 2013). Additionally, anti-inflammatory-dose aspirin is recommended during the disease’s acute phase, with a reduction of dosage as the inflammation and fever subside (Eleftheriou, 2013). In case of IVIG resistance in the patient, corticosteroids are the recommended alternative course of treatment (Eleftheriou, 2013). During treatment and diagnosis, additional tests should be performed to monitor the inflammatory process associated with KD. Specifically, increased erythrocyte sedimentation rate, C-reactive protein, and platelet count are strong indicators of KD (McCrindle et al., 2017). Electrocardiography should also be performed to assess mitral regurgitation or other cardiac anomalies (McCrindle et al., 2017). This monitoring is especially important in KD, considering the absence of a specific test.
As a disease that primarily affects young children, but whose long-term effects can linger into adulthood, KD makes collaboration between pediatric specialists and adult care specialists critical. After the symptoms and clinical signs are resolved, long-term management of the illness focuses on surveillance of the patient’s cardoivascular health to prevent thrombosis and myocardial ischemia (McCrindle et al., 2017). The specifics of this long-term surveillance depend on the presence and severity of the patient’s coronary artery involvement (McCrindle, 2017). Follow-up cardiovascular risk factor assessments should generally be performed yearly, with additional assessment for ischemia in cases where an aneurysm is present (McCrindle et al.2017). These measures, as well as physical activity and reproductive counseling are aimed at preventing cardoivascular complications later in the patient’s life.
References
Eleftheriou, D., Levin, M., Shingadia, D., Tulloh, R., Klein, N., & Brogan, P. (2013). Management of Kawasaki disease. Archives of Disease in Childhood, 99(1), 74–83.
McCrindle, B. W., Rowley, A. H., Newburger, J. W., Burns, J. C., Bolger, A. F., Gewitz, M., … Pahl, E. (2017). Diagnosis, treatment, and long-term management of kawasaki disease: A scientific statement for health professionals from the American Heart Association. Circulation, 135(17), e927–e999.
Patel, R. M., & Shulman, S. T. (2015). Kawasaki disease: a comprehensive review of treatment options. Journal of Clinical Pharmacy and Therapeutics, 40(6), 620–625.