Kawasaki Disease

Gooty, Vasu D.; Sharma, Kavita; Hussain, Tarique

Kawasaki disease (KD) is an acute vasculitis leading to coronary artery dilation and aneurysm in untreated patients. Etiology includes infectious triggers, autoimmune response, genetically susceptible host response, windborne agents. Diagnosis of classical KD is made based on the presence of ≥5 days of fever and presence of ≥4 of the 5 principal clinical features, which include conjunctival injection, oropharynx changes, peripheral extremity changes, diffuse erythematous rash, cervical lymphadenopathy. In addition, serum markers of acute inflammation such CRP and ESR are also elevated. Patients without sufficient clinical findings are known to have incomplete or atypical KD. Prompt diagnosis is essential, and treatment should be initiated with intravenous Immunoglobulin (IVIG). Early detection and follow-up of evolving coronary artery changes should be performed by serial echocardiograms and appropriate thromboprophylaxis should be initiated in a timely fashion. Risk stratification for long-term management is based primarily on coronary changes. Patients with coronary aneurysms require lifelong cardiology follow-up. In the current COVID-19 pandemic caused by SARS-CoV-2 virus, pediatric patients may present with atypical KD or acute toxic shock like features called Multisystem Inflammatory Syndrome in Children (MIS-C). Most of them present with myocardial dysfunction and gastrointestinal symptoms in addition to atypical KD features. Although, the true mechanism is not completely understood, the proposed hypothesis for myocardial injury is due to “cytokine storm” from the proinflammatory and regulatory T cells. Early recognition and initiation of IVIG is crucial in addition to ongoing intensive care management. Long-term outcomes are yet to be determined.