Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease

TLDR

Kawasaki disease is an acute, self‑limited vasculitis of childhood presenting with fever, conjunctivitis, mucosal changes, rash, and lymphadenopathy, and untreated coronary artery aneurysms develop in 15–25 % of patients, risking ischemic heart disease or sudden death. The authors propose a new algorithm to help clinicians decide when to perform echocardiography, administer intravenous immunoglobulin, or both for children with ≥5 days of fever and ≤4 classic criteria. A multidisciplinary committee revised the American Heart Association guidelines, reviewing evidence for initial IVIG therapy, retreatment options such as corticosteroids, TNF‑α antagonists, and abciximab, and outlining risk‑stratified long‑term management including antiplatelet/anticoagulant therapy, activity restrictions, and follow‑up diagnostics. The updated recommendations provide clinicians with a range of acceptable approaches for initial evaluation, acute treatment, and long‑term management of Kawasaki disease.

Abstract

Background— Kawasaki disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in ∼15% to 25% of untreated children and may lead to ischemic heart disease or sudden death. Methods and Results— A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki disease. The writing group proposes a new algorithm to aid clinicians in deciding which children with fever for ≥5 days and ≤4 classic criteria should undergo echocardiography, receive intravenous gamma globulin (IVIG) treatment, or both for Kawasaki disease. The writing group reviews the available data regarding the initial treatment for children with acute Kawasaki disease, as well for those who have persistent or recrudescent fever despite initial therapy with IVIG, including IVIG retreatment and treatment with corticosteroids, tumor necrosis factor-α antagonists, and abciximab. Long-term management of patients with Kawasaki disease is tailored to the degree of coronary involvement; recommendations regarding antiplatelet and anticoagulant therapy, physical activity, follow-up assessment, and the appropriate diagnostic procedures to evaluate cardiac disease are classified according to risk strata. Conclusions— Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients with Kawasaki disease. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients.