Adult-onset Kawasaki disease (mucocutaneous lymph node syndrome) and concurrent Coxsackievirus A4 infection: a case report

Ueda, Yuki; Kenzaka, Tsuneaki; Noda, Ayako; Yamamoto, Yu; Matsumura, Masami

It is important not only to manage symptoms in the acute phase of KD but also to prevent the cardiovascular after-effects. There are three essential treatments for KD: 1) intravenous immunoglobulin (IVIG) to obtain an anti-inflammatory effect, 2) aspirin for anti-inflammatory and antiplatelet effects, and 3) management of any complications (eg, meningitis or disseminated intravascular coagulation). IVIG can mitigate inflammation and the coronary artery complications. A dose of 2 g/kg of IVIG in a single infusion has been found to be effective. This therapy should be initiated within the first 10 days of illness. 14,15 IVIG has not been demonstrated to be effective if administered after the first 10 days of illness. Furthermore, one report has noted a risk of unfavorable effects with IVIG regarding cardiac sequelae if IVIG is started on day 9 or later. 16 In our patient, IVIG was not administered because he was admitted to our hospital on the 13th day of illness. Aspirin is often administered with IVIG in patients with KD. In the acute phase, the American Heart Association recommends high-dose aspirin (80-100 mg/kg/day) to achieve an anti-inflammatory effect. 14 However, it is not clear if a higher dose would be more effective than the lower dose (30-50 mg/ kg/day). 17 When the patient remains afebrile for 48-72 hours, the aspirin dose is lowered (3-5 mg/kg/day) and maintained to achieve an antiplatelet effect until the patient shows no evidence of coronary changes for 6-8 weeks after onset. 14 In addition, regular echocardiographic evaluation should be performed to assess for the possibility of coronary artery aneurysm.