These characteristic clinical features of ≥ 5 days of fever, bilateral non-purulent conjunctivitis, rash, cervical lymphadenopathy, and mucocutaneous changes remain the mainstay of diagnosis of KD. Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome, is an acute self-limited vasculitis of unknown etiology with characteristic clinical features first described in Japanese children in 1967. Further investigation is warranted to further delineate these conditions and optimize treatment of these conditions given the ongoing COVID-19 pandemic.

A significant proportion of children diagnosed with MIS-C, a post-infectious syndrome of SARS-CoV-2 infection, meet criteria for Kawasaki disease. The 2017 American Heart Association (AHA) treatment guidelines have included changes in aspirin dosing (including both 80–100 mg/kg/day and 30–50 mg/kg/day treatment options), consideration of the use of adjuvant corticosteroid therapy in patients at high risk of IVIG resistance, and the change in steroid regimen for refractory KD to include both pulse-dose IVMP and longer course of prednisolone with an oral taper. MIS-C with Kawasaki manifestations is more commonly seen in children < 5 years of age. MIS-C is a clinically distinct entity from KD with broad clinical manifestations and multiorgan involvement (cardiac, GI, hematologic, dermatologic, respiratory, renal). The efficacy of other agents (atorvastatin, doxycycline) is currently being investigated. Additional adjunctive therapies (etanercept, infliximab, cyclosporin) may also provide limited benefit, but data is limited to single studies and subgroups of patients with cardiac abnormalities. Recent FindingsĪdjunctive corticosteroid therapy has been shown to reduce the rate of coronary artery dilation in children at high risk for IVIG resistance in multiple Japanese clinical studies (most notably RAISE study group). Provide an updated review of the clinical management and diagnosis of Kawasaki disease with inclusion of potential diagnostic difficulties with multisystem inflammatory syndrome in children (MIS-C) given the ongoing COVID-19 pandemic.

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