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Kawasaki disease (ICD-10 code: M30.3) is a common self-limiting childhood febrile illness classified as an acute, systemic necrotizing vasculitis of unknown etiology that is thought to be related to infectious etiology due to its clinical manifestations such as fever and lymphadenopathy (McCrindle et al., 2017). Kawasaki disease more commonly occurs in Asian patients (McCrindle et al, 2017). Kawasaki disease is more common in the male sex when compared to female counterparts and most often affects children under the age of 5 (McCrindle et al., 2017). Kawasaki disease is also a leading cause of coronary artery disease which can occur in up to 20-25% of children under the age of 5 and can lead to myocardial infarction and cardiac arrhythmias if complications occur (McCrindle et al, 2017).
The pathophysiology of Kawasaki is not exactly known (McCrindle et al, 2017). However, theories believe disease begins with an infection in the body such as strep throat and upper respiratory infections (McCrindle et al, 2017). As a result of infection, an intense inflammatory cell response similar to sepsis throughout the body occurs which also includes the coronary arteries (McCrindle et al, 2017). During the inflammatory response, neutrophils infiltrate the coronary artery wall leading to the production of matrix metalloproteinases (McCrindle et al., 2017). As a result of this inflammatory response the coronary arteries in the heart undergo damage to their collagen and elastic fibers which disturbs the integrity of the vessel wall and eventually leads to aneurysm formation (McCrindle et al., 2017).
The key clinical features of Kawasaki disease include fever for 5 days, erythema and edema in the hands and feet, desquamation of the fingertips after 2-3 weeks along with a polymorphous rash that originates in the trunk of the body (McCrindle et al., 2017). Children will also present with bilateral conjunctivitis, mucositis, and a classic swollen “strawberry tongue (McCrindle et al., 2017). Cervical lymphadenopathy may also be present (McCrindle et al., 2017). Patients may also present with cardiovascular findings such as myocarditis, pericarditis, and coronary artery abnormalities (McCrindle et al., 2017).
Initial diagnostics that can be done in Kawasaki disease include an ESR, CRP, CBC, CMP, and an echocardiogram (McCrindle et al., 2017). ESR and CRP inflammatory markers may be elevated upon result (McCrindle et al., 2017). A CBC may show an elevated WBC count, anemia, and thrombocytosis (McCrindle et al., 2017). An echocardiogram will be useful in assessing a patient for coronary artery structural damage (McCrindle et al., 2017). If a patient is found to have a notable aneurysm in the coronary artery, cardiac catheterization, and angiography may be done (McCrindle et al., 2017).
Treatment for Kawasaki disease includes IVIG 2g/kg preferably during the first 10 days, low-dose oral aspirin 3-5mg/kg/day for 6 weeks, and systemic glucocorticoids (McCrindle et al., 2017). Kawasaki disease is one of the only exceptions in which children may be given aspirin due to the risk of developing Reye syndrome (McCrindle et al., 2017). Aspirin provides antiplatelet effects and helps prevent coronary aneurysms in Kawasaki disease (McCrindle et al., 2017). Parents and patients should be instructed to avoid contact sports such as martial arts and football while patients are on blood thinning agents (McCrindle et al., 2017).
Upon diagnosing a patient with Kawasaki disease, patients can often be sent home on oral aspirin and referred to a cardiologist to be closely monitored for complications (McCrindle et al., 2017). Unstable or high-risk patients will require hospitalization but this is often not the case since Kawasaki disease is often self-limiting (McCrindle et al., 2017). Complications of Kawasaki disease include arthritis, acute coronary syndrome, coronary thrombosis, aseptic meningitis, and valvulitis (McCrindle et al., 2017). Parents should be instructed to call their child’s pediatrician or take them to the nearest ER if the patient starts complaining of severe chest pain (McCrindle et al., 2017). A cardiologist may also perform an echocardiogram within a few weeks to check for any cardiac abnormalities (McCrindle et al., 2017). Patients should be closely followed for long-term complications of Kawasaki disease which include coronary thrombosis, acute coronary syndrome, bowel ischemia, and rupture of giant coronary artery aneurysms (McCrindle et al., 2017).
References
McCrindle, B. W., Rowley, A. H., Newburger, J. W., Burns, J. C., Bolger, A. F., Gewitz, M., Baker, A. L., Jackson, M., Takahashi, M., Shah, P. B., Kobayashi, T., Wu, M.-H., Saji, T. T., & 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). https://doi.org/10.1161/cir.0000000000000484
